Rath W, Loos W, Kuhn W
Universitäts-Frauenklinik Göttingen.
Zentralbl Gynakol. 1994;116(4):195-201.
The HELLP syndrome is a severe and life-threatening complication of preeclampsia with typical laboratory findings. The frequency of the disease is 1 to 150-300 live births in perinatal centers. The median gestational age at presentation is 34 weeks, however, the disease may also develop during the early postpartum period. Right upper quadrant pain is the most striking clinical symptom; in up to 15% of cases neither hypertension nor proteinuria is present on admission. For the detection of hemolysis determination of haptoglobin levels is the most suitable method. Coagulation disorders are more pronounced in patients with the HELLP syndrome as compared to those with preeclampsia without the HELLP constellation, however the full-blown syndrome of disseminated intravascular coagulation (DIC) is not a leading symptom but the consequence of delayed diagnosis and/or therapy of the primary disease. The course of the HELLP syndrome is unpredictable. On the one hand, complete reversal of symptoms under conservative treatment have been reported in individual cases, on the other hand, rapid, therapy-resistant deterioration of the disease had been observed in the majority of patients accompanied by severe complications (e.g. liver rupture). As a consequence the mother and the newborn need intensive care, and these women should be delivered in an obstetric intensive care unit. The maternal mortality reported from the international literature is 3.3%, and the perinatal mortality 22.6%.(ABSTRACT TRUNCATED AT 250 WORDS)
HELLP综合征是先兆子痫的一种严重且危及生命的并发症，具有典型的实验室检查结果。在围产中心，该病的发病率为每150 - 300例活产中有1例。发病时的中位孕周为34周，不过，该病也可能在产后早期发生。右上腹疼痛是最显著的临床症状；高达15%的病例入院时既无高血压也无蛋白尿。对于溶血的检测，测定触珠蛋白水平是最合适的方法。与无HELLP综合征的先兆子痫患者相比，HELLP综合征患者的凝血障碍更为明显，然而，弥漫性血管内凝血（DIC）的典型综合征并非主要症状，而是原发性疾病诊断和/或治疗延迟的后果。HELLP综合征的病程不可预测。一方面，个别病例报告了保守治疗下症状完全缓解的情况，另一方面，大多数患者出现了疾病迅速、抗治疗的恶化，并伴有严重并发症（如肝破裂）。因此，母亲和新生儿需要重症监护，这些女性应在产科重症监护病房分娩。国际文献报道的孕产妇死亡率为3.3%，围产儿死亡率为22.6%。（摘要截取自250字）
