Barrou Z, Billaud L, Guilhaume B, Bertagna X, Luton J P
Clinique des Maladies endocriniennes et métaboliques, Hôpital Cochin, Paris.
Presse Med. 1990 Dec 1;19(41):1883-6.
The presence of adrenal nodules in patients with congenital adrenal hyperplasia seems to be relatively frequent but is seldom reported. We observed such nodular formations in 3 women and 2 men aged from 19 to 71 years. Four patients had enzyme deficiency revealed in childhood (3 were deficient in 21-hydroxylase and 1 in 11-hydroxylase); the fifth patient had a virilizing form of an unrecognized 21-hydroxylase deficiency. In 3 patients the nodular formations had a diameter of 10 millimetres or less. In the other 2 patients the diameter was greater than 30 millimetres, and an adrenocortical adenoma was found at surgery. In one case a preoperative increase of medical treatment resulted in partial regression of the nodules. Our findings show that young subjects may have adrenal nodules, and their dependence on ACTH is discussed. We also discuss the possible influence of these nodules on the difficulty of medical treatment, the need for a life-long therapy and the necessity to look for a congenital adrenal hyperplasia when confronted with an "incidentaloma".
先天性肾上腺增生患者中肾上腺结节的存在似乎相对常见,但鲜有报道。我们观察到3名女性和2名男性(年龄在19至71岁之间)出现了此类结节形成。4名患者在儿童期被发现存在酶缺乏(3名缺乏21-羟化酶,1名缺乏11-羟化酶);第5名患者患有未被识别的21-羟化酶缺乏的男性化形式。3名患者的结节形成直径为10毫米或更小。另外2名患者的直径大于30毫米,手术时发现为肾上腺皮质腺瘤。在1例中,术前增加药物治疗导致结节部分消退。我们的研究结果表明,年轻受试者可能出现肾上腺结节,并讨论了它们对促肾上腺皮质激素(ACTH)的依赖性。我们还讨论了这些结节对治疗难度、终身治疗需求的可能影响,以及面对“偶发瘤”时寻找先天性肾上腺增生的必要性。