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首页> 外文期刊>European journal of endocrinology >ENDOCRINE DISORDERS IN PREGNANCY: Pheochromocytoma and pregnancy: a deceptive connection
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ENDOCRINE DISORDERS IN PREGNANCY: Pheochromocytoma and pregnancy: a deceptive connection

机译:内分泌疾病:嗜铬细胞瘤和妊娠:欺骗性联系

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A pheochromocytoma in a pregnant patient is one of the most threatening medical conditions for mother, fetus, and physician. Although extraordinarily rare with a frequency of 0.002% of all pregnancies, this tumor is notorious for its devastating consequences. As in non-pregnant patients, the signs and symptoms are quite variable but not specific, with hypertension being one of the most prominent signs. Confusion with the much more prevalent forms of pregnancy-related hypertension is the main cause of overlooking the diagnosis. If undiagnosed, maternal and fetal mortality is around 50%. Conversely, early detection and proper treatment during pregnancy decrease the maternal and fetal mortality to <5 and 15% respectively. For the biochemical diagnosis, plasma or urinary metanephrines are the tests of first choice since they have a nearly maximal negative predictive value. For reliable localization, only magnetic resonance imaging is suitable, with a sensitivity of more than 90%. When the tumor is diagnosed in the first 24 weeks of gestation, it should be removed by laparoscopic adrenalectomy after 10–14 days of medical preparation with the same drugs as in non-pregnant patients. If the tumor is diagnosed in the third trimester, the patient should be managed until the fetus is viable using the same drug regimen as for regular surgical preparation. Cesarean section with tumor removal in the same session or at a later stage is then preferred since vaginal delivery is possibly associated with higher mortality. Despite all technical diagnostic and therapeutic progress over the last decades, the key factor for further reduction of maternal and fetal mortality is early awareness and recognition of the potential presence of a pheochromocytoma in a pregnant patient with hypertension.
机译:孕妇的嗜铬细胞瘤是母亲,胎儿和医师面临的最危险的医学疾病之一。尽管异常罕见,占所有妊娠的0.002%,但该肿瘤因其破坏性后果而臭名昭著。与未怀孕的患者一样,体征和症状变化很大,但并不特异,高血压是最突出的体征之一。与更常见的妊娠相关性高血压相混淆是忽视诊断的主要原因。如果不确诊,孕产妇和胎儿的死亡率约为50%。相反,怀孕期间的早期发现和适当的治疗将孕产妇和胎儿的死亡率分别降低到<5%和15%。对于生化诊断,血浆或尿中的肾上腺素是几乎首选的阴性预测值,因此是首选测试。对于可靠的定位,仅磁共振成像适合,其灵敏度超过90%。当在妊娠的最初24周内诊断出肿瘤时,应在药物准备10-14天后使用与未怀孕患者相同的药物通过腹腔镜肾上腺切除术将其清除。如果在妊娠中期诊断出肿瘤,则应使用与常规手术准备相同的药物治疗患者直至胎儿存活。由于阴道分娩可能会导致更高的死亡率,因此优选在同一疗程中或在较晚阶段切除剖腹产的剖宫产术。尽管过去几十年来在技术诊断和治疗上取得了所有进展,但进一步降低孕产妇和胎儿死亡率的关键因素是早期认识和认识到高血压孕妇中嗜铬细胞瘤的潜在存在。

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