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1型神经纤维瘤病合并妊娠期间的嗜铬细胞瘤

Pheochromocytoma in neurofibromatosis type 1 during pregnancy.

作者信息

Remón-Ruiz Pablo, Aliaga-Verdugo Alberto, Guerrero-Vázquez Raquel

机构信息

a Department of Endocrinology and Nutrition , Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Rocío/CSIC/Universidad de Sevilla , Seville , Spain.

出版信息

Gynecol Endocrinol. 2017 Feb;33(2):93-95. doi: 10.1080/09513590.2016.1254181. Epub 2016 Dec 2.

Abstract

Pregnant women with neurofibromatosis type 1 (NF-1) have increased complications during gestation, including hypertensive disorders that are sometimes caused by pheochromocytoma. Pheochromocytoma is an extremely rare condition during pregnancy, and the main clinical manifestation is hypertension. If not properly treated, pheochromocytoma has high maternal and fetal mortality rates. Early recognition and adequate clinical management before delivery have led to better outcomes in the last few decades. Despite the association of NF-1 and pheochromocytoma, there are few clinical reports of these two conditions in pregnant patients. We present a rare case of pheochromocytoma diagnosed during pregnancy in a patient with NF-1, and we describe the treatment and the obstetric and fetal outcomes. We also review other medical conditions related to NF-1 that complicated this patient's pregnancy.

摘要

患有1型神经纤维瘤病(NF-1)的孕妇在妊娠期并发症增多,包括有时由嗜铬细胞瘤引起的高血压疾病。嗜铬细胞瘤在孕期极为罕见,主要临床表现为高血压。若治疗不当,嗜铬细胞瘤会导致母婴死亡率很高。在过去几十年中,分娩前的早期识别和充分的临床管理带来了更好的结局。尽管NF-1与嗜铬细胞瘤有关联,但关于这两种病症在妊娠患者中的临床报告很少。我们报告了1例在孕期诊断出嗜铬细胞瘤的NF-1患者的罕见病例,并描述了其治疗情况以及产科和胎儿结局。我们还回顾了与该患者妊娠相关的其他合并症。

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