Department of Obstetrics and Gynecology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA.
Department of Obstetrics and Gynecology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA.
Am J Obstet Gynecol. 2023 Nov;229(5):502-515.e10. doi: 10.1016/j.ajog.2023.04.046. Epub 2023 May 5.
We performed a systematic review to evaluate the clinical presentation and maternal and fetal outcomes in pregnancies with early-onset HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome.
PubMed, Ovid MEDLINE, Scopus, CINAHL, Cochrane Library, and ClinicalTrials.gov were queried from inception through January 1, 2023 with the following terms: "HELLP syndrome," "HELLP," "hemolysis, elevated liver enzymes, low platelets," "hemolysis, elevated liver enzymes, low platelets syndrome," "pre-viable," "peri-viable," "previable," "periviable," "first trimester," "second trimester," "before 23 weeks," "<23 weeks," "<23 week gestation," and "before 23 weeks gestation." We also included an additional case from our institution.
Abstracts, unpublished studies, and review articles were excluded, yielding 46 studies that met our inclusion criteria.
Two reviewers (N.S.I. and M.H.M.) performed the study selection and subsequent data extraction independently, after which the results were reviewed together. PRISMA guidelines were followed, and our study was registered at PROSPERO (CRD42021292692).
A total of 55 patients had 58 pregnancies complicated by early-onset HELLP syndrome, including 3 with recurrent HELLP. The most common presenting signs/symptoms were abdominal pain (35/45; 78%), hypertension (32/49; 65%), nausea/vomiting (16/45; 36%), headache (13/45; 29%), and edema (8/45; 18%). Lactate dehydrogenase ≥600 IU/L was observed in 21 of 31 (68%) cases, whereas liver enzyme abnormalities and thrombocytopenia were reported in 48 of 51 (94%) and 50 of 54 (93%) cases, respectively. Maternal complications were encountered in 25 of 56 (45%) cases. The most common complications were hepatic (13/56; 23%), central nervous system-related (11/56; 20%), and respiratory (11/56; 20%). In 36 of 57 (63%) cases, pregnancy was terminated. Of the 21 continued pregnancies, early fetal death (at <20 weeks' gestation) was reported in 10 (48%), stillbirth in 6 (28%), and neonatal demise in 2 (10%). Living neonates were reported in 3 of 21 (14%) cases, all delivered at 23 weeks. The perinatal mortality rate was 73% (8/11). One case (2%) reported maternal death. Antiphospholipid syndrome was diagnosed in 14 of 29 (48%) cases.
Early-onset HELLP syndrome presents with symptoms similar to those observed in later gestation. Maternal complications are life-threatening, with the most common complications being hepatic, central nervous system-related, and respiratory. Fetal outcomes are poor.
我们进行了一项系统评价，以评估早发型 HELLP（溶血、肝酶升高和血小板减少）综合征孕妇的临床表现以及母婴结局。
从建库至 2023 年 1 月 1 日，我们在 PubMed、Ovid MEDLINE、Scopus、CINAHL、 Cochrane Library 和 ClinicalTrials.gov 中使用以下术语进行了检索：“HELLP 综合征”、“HELLP”、“溶血、肝酶升高、血小板减少”、“溶血、肝酶升高、血小板减少综合征”、“极早产”、“围产可存活”、“极早产”、“围产可存活”、“孕早期”、“孕中期”、“<23 周”、“<23 周”、“<23 孕周”和“<23 孕周”。我们还纳入了来自本机构的另外 1 例病例。
排除摘要、未发表的研究和综述文章，最终纳入 46 项符合我们纳入标准的研究。我们遵循了 PRISMA 指南，并在 PROSPERO（CRD42021292692）上注册了我们的研究。
共有 55 例患者的 58 例妊娠并发早发型 HELLP 综合征，其中 3 例为复发性 HELLP。最常见的表现为腹痛（35/45；78%）、高血压（32/49；65%）、恶心/呕吐（16/45；36%）、头痛（13/45；29%）和水肿（8/45；18%）。31 例（68%）中观察到乳酸脱氢酶≥600IU/L，48 例（94%）和 50 例（93%）分别报告肝酶异常和血小板减少。56 例（45%）中出现了母体并发症。最常见的并发症为肝脏（13/56；23%）、中枢神经系统相关（11/56；20%）和呼吸系统（11/56；20%）。36 例（63%）中终止了妊娠。在 21 例继续妊娠中，报告了 10 例（48%）早期胎儿死亡（<20 周妊娠）、6 例（28%）死产和 2 例（10%）新生儿死亡。21 例中有 3 例（14%）存活新生儿出生，均在 23 周时分娩。围生儿死亡率为 73%（8/11）。1 例（2%）报告了产妇死亡。29 例中有 14 例（48%）诊断为抗磷脂综合征。
早发型 HELLP 综合征表现出与晚期妊娠相似的症状。母体并发症具有生命威胁，最常见的并发症为肝脏、中枢神经系统相关和呼吸系统。胎儿结局较差。
