XXXX in pregnancy: a review of the Japanese literature.

@entity1 @entity101 @entity1911 @entity5 @entity6064
We review the 35 cases with @entity1911 associated with pregnancy including our own 3 cases in the Japanese literature from 1958 to 1993. In a total of 35 cases, the overall maternal mortality rate was 11% and the fetal loss 22%. Recently, after 1981, the maternal mortality rate and the fetal loss were significantly reduced to one @entity1 (6%). Although antepartum diagnosis of @entity1911 was made in only 32% of the @entity1 , maternal mortality was reduced to zero and fetal loss to 18%. An initial diagnosis of @entity6064 of pregnancy was made in 17 @entity1 (49%) and of @entity1911 in 10 @entity1 (29%). A diagnosis of @entity1911 was made before delivery in 11 @entity1 (31%), after delivery in 22 @entity1 (63%), at autopsy in one @entity1 (3%), and unknown in one @entity1 (3%). The symptoms mostly suggestive of @entity1911 were paroxysmal @entity101 in 27 (77%) of the 35 @entity1 . The clinical symptoms generally appeared in the third trimester in 23 @entity1 (66%). @entity1911 is a rare disease, but a high index of clinical suspicion must be kept in mind and @entity1911 must be listed in the differential diagnosis of @entity101 associated with pregnancy. Recently, abdominal ultrasound study and MR imaging can be used to localize the @entity5 during the antepartum period. Once the diagnosis is confirmed, alpha-blockade is essential and beta-blockade may be required. In the first and second trimesters, @entity5 resection has a good fetal outcome; in later pregnancy, delivery by elective caesarean section followed by @entity5 resection is recommended.
