Lubin Vanessa, Gautier Jean-François, Antoine Jean-Marie, Beressi Jean-Paul, Vexiau Patrick
Service d'endocrinologie et nutrition et maladies métaboliques, Hôpital Saint-Louis, 1, avenue Claude Vellefaux, 75010 Paris.
Presse Med. 2002 Nov 9;31(36):1706-13.
The rare association of Cushing's syndrome and pregnancy is explained by the amenorrhea and sterility inherent to the syndrome. In the literature, 125 cases have been reported: 30 cases of early diagnosis and 95 others diagnosed in the second half of pregnancy.
When hypercorticism exists before pregnancy it is hardly secretory. Its diagnosis, at an early stage, is not hindered by the hormone modifications of pregnancy. Its aetiological treatment raises the problem of the compatibility in pursuing the latter.
The positive and aetiological diagnoses of Cushing's syndrome are difficult and its prevalence may therefore be underestimated. The evocative clinical signs are unspecific: excessive weight gain, hypertension of pregnancy and gestational diabetes. The 24-hour free hypercortisoluria and the absence of dexamethasone inhibition are of little diagnostic value after the 14th week of amenorrhea. The positive diagnosis therefore relies essentially on the abolition of the circadian rhythm of cortisol. The biological hyperandrogenia commonly observed is not discriminating. Adrenal aetiologies are frequent. Imaging must be performed to eliminate an adrenocortical tumor.
The maternal prognosis depends on the hypertension, preeclampsia, diabetes and the complications of Cushing's syndrome. It depends on the activity of the hypercorticism and its early aetiological treatment, which must not be delayed after pregnancy. The foetal prognosis depends on the maternal prognosis. It is represented by preterm delivery, hypotrophy and death of the foetus in utero. The therapeutic management must be symptomatic and aetiologic, maternal and obstetrical.
库欣综合征与妊娠的罕见关联可由该综合征固有的闭经和不育来解释。文献中已报道125例:30例为早期诊断,另外95例在妊娠后半期诊断。
若妊娠前存在皮质醇增多症,其分泌几乎不受影响。早期诊断不受妊娠激素变化的阻碍。其病因治疗引发了继续治疗时的兼容性问题。
库欣综合征的阳性和病因诊断困难,因此其患病率可能被低估。提示性临床体征不具特异性:体重过度增加、妊娠高血压和妊娠期糖尿病。闭经14周后,24小时尿游离皮质醇增多及地塞米松抑制试验阴性的诊断价值不大。因此,阳性诊断主要依赖于皮质醇昼夜节律的消失。常见的生物学高雄激素血症不具鉴别意义。肾上腺病因较为常见。必须进行影像学检查以排除肾上腺皮质肿瘤。
母亲的预后取决于高血压、先兆子痫、糖尿病及库欣综合征的并发症。它取决于皮质醇增多症的活动情况及其早期病因治疗,妊娠后不得延迟治疗。胎儿的预后取决于母亲的预后。表现为早产、胎儿生长受限及胎儿宫内死亡。治疗管理必须是对症和病因性的,包括母体和产科方面。