单卵双胞胎中的不一致性先天性巨结肠病。

Discordant Hirschsprung's disease in monozygotic twins.

作者信息

Siplovich L, Carmi R, Bar-Ziv J, Karplus M, Mares A J

出版信息

J Pediatr Surg. 1983 Oct;18(5):639-40. doi: 10.1016/s0022-3468(83)80383-2.

Abstract

Two pairs of twins are being reported where monozygocity was supported by the observed sharing of a single and common placenta and by ABO and HLA identity. Only one of each pair of twins was affected by long-segment Hirschsprung's disease; the other twins were entirely normal. Discordant Hirschsprung's disease in monozygotic twins is very rare. The etiology in such cases is influenced by genetic or by environmental intrauterine factors rather than by postnatal influences, as suggested elsewhere.

摘要

据报道，有两对双胞胎，单胎盘的共享以及ABO和HLA血型相同支持了它们的单合子性。每对双胞胎中只有一个患有长段先天性巨结肠病；另一个双胞胎完全正常。单合子双胞胎中先天性巨结肠病不一致的情况非常罕见。正如其他地方所指出的，此类病例的病因受遗传或子宫内环境因素影响，而非出生后影响。