Management of a multiple endocrine neoplasia type 1 during pregnancy: a case with primary hypertension

Abstract

Multiple endocrine neoplasia type 1 (MEN 1) is a rare hereditary disease which transmitted as autosomal dominant disorder with high penetrance. MEN1 includes vary combinations of more than 20 endocrine and non-endocrine tumors. Clinically, MEN1 is characterized by tumor or hyperplasia in two or more endocrine tissues (parathyroid, pituitary, pancreas, adrenal glands). Since it is a rare condition, there are no guidelines with respect to the follow-up of pregnant women with MEN 1, here we the first Asian case of a 32-year-old primigravida with a confirmed diagnosis of MEN1 and primary hypertension prior to conception, cesarean section was performed at 34⁺²weeks for severe preeclampsia and potential risks, both the patient and fetus recovered well.

妊娠期多发性内分泌腺瘤病I型的处理：1例原发性高血压患者 摘要

多发性内分泌腺瘤病I型（MEN1）是一种罕见的遗传性疾病, 外显率高的常染色体显性遗传性疾病。MEN 1包括20多种内分泌和非内分泌肿瘤的不同组合。MEN 1的临床特征是两个或两个以上内分泌组织（甲状旁腺、垂体、胰腺、肾上腺）的肿瘤或增生。由于这是一种罕见的情况, 没有关于MEN 1孕妇随访的指南, 我们报道的是亚洲第一例32岁的初产妇, 在受孕前被确诊为MEN 1和原发性高血压, 由于严重的子痫前期和潜在的风险, 在34⁺²周时进行了剖宫产手术, 患者和胎儿都恢复得很好。

Keywords:

• Endocrinology
• pregnancy
• maternal–fetal medicine

Disclosure statement

The authors do not have a conflict of interest.