Yawar Aasima, Zuberi Lubna M, Haque Naeemul
The Department of Endocrinology and Metabolism, Aga Khan University Hospital, Karachi, Sindh, Pakistan.
Endocr Pract. 2007 May-Jun;13(3):296-9. doi: 10.4158/EP.13.3.296.
To describe a patient with untreated Cushing's disease who had 2 spontaneous pregnancies that resulted in healthy babies on both occasions.
We present a case report with clinical, laboratory, and imaging data and discuss the literature pertaining to pregnancy in patients with Cushing's syndrome.
A 28-year-old woman came to our endocrinology clinic with a 1-year history of symptoms and signs of Cushing's syndrome. An elevated 24-hour urinary cortisol excretion and an unsuppressed 1-mg overnight dexamethasone test confirmed the diagnosis. On her next visit, she reported a confirmed pregnancy, which ultimately resulted in the birth of a normal child. Further work-up subsequently showed 2 elevated 24-hour urinary cortisol values, loss of diurnal variation, and an elevated corticotropin level. There was lack of suppression on low-dose and high-dose overnight dexamethasone suppression tests. Magnetic resonance imaging of the pituitary showed normal findings. Inferior petrosal sinus sampling was recommended, but she declined the procedure. The patient returned 3 years later for reevaluation, at which time she reported the birth of another healthy child by cesarean delivery 10 months previously. There were no reported maternal or fetal complications. Examination at this visit revealed buccal pigmentation and proximal myopathy. Investigations showed increased 24-hour urinary cortisol excretion and serum corticotropin levels. Repeated magnetic resonance imaging disclosed a microadenoma on the right side of the pituitary. Unstimulated inferior petrosal sinus sampling showed a gradient to the right; thus, the presence of pituitary-dependent Cushing's disease was confirmed.
Our case demonstrates that patients with pituitary-dependent Cushing's disease are more likely to have spontaneous pregnancies with favorable outcomes than are patients with Cushing's syndrome due to other causes. Our patient, despite having Cushing's disease for more than 7 years, had 2 uneventful pregnancies that produced normal healthy children, without exacerbation of her disease during pregnancy.
描述一名未经治疗的库欣病患者，该患者有两次自然妊娠，两次均产下健康婴儿。
我们呈现一份包含临床、实验室及影像学数据的病例报告，并讨论与库欣综合征患者妊娠相关的文献。
一名28岁女性因有1年库欣综合征的症状和体征前来我们的内分泌科门诊。24小时尿皮质醇排泄量升高以及1毫克过夜地塞米松试验未被抑制，证实了诊断。在她的下一次就诊时，她报告已确诊怀孕，最终产下一名正常婴儿。进一步检查随后显示24小时尿皮质醇值两次升高、昼夜节律消失以及促肾上腺皮质激素水平升高。低剂量和高剂量过夜地塞米松抑制试验均无抑制作用。垂体磁共振成像显示结果正常。建议进行岩下窦取样，但她拒绝了该检查。3年后该患者回来复诊，此时她报告10个月前经剖宫产又产下一名健康婴儿。未报告有母体或胎儿并发症。此次就诊检查发现颊部色素沉着和近端肌病。检查显示24小时尿皮质醇排泄量增加以及血清促肾上腺皮质激素水平升高。重复的磁共振成像显示垂体右侧有一个微腺瘤。未刺激状态下的岩下窦取样显示右侧有梯度变化；因此，垂体依赖性库欣病得以确诊。
我们的病例表明，与其他原因导致的库欣综合征患者相比，垂体依赖性库欣病患者更有可能自然妊娠并获得良好结局。我们的患者尽管患有库欣病超过7年，但有两次顺利的妊娠，产下正常健康的婴儿，且孕期病情未加重。
