1/51. Cushing's syndrome in pregnancy treated by ketoconazole: case report and review of the literature.We report on a 30-year-old female with a pituitary-dependent Cushing's disease, who refused transsphenoidal surgery and was treated with ketoconazole and cabergoline. After approximately 3 years of therapy, the patient herself decided, without the knowledge of her treating physician, to interrupt contraception. As the patient became pregnant she ceased the intake of all medication (between the third and seventh week), but resumed it soon after pregnancy was diagnosed because of relapsing clinical signs. pregnancy and vaginal delivery at 37 weeks gestation passed uneventfully. The newborn male infant did not demonstrate any congenital malformations and was normally sexually developed. With reference to this case, we discuss the difficulties in the medical treatment of Cushing's syndrome during pregnancy. Whereas outside pregnancy only efficacy and side-effects are taken into account, teratogenicity is an important question in these patients. Experience with different drugs is listed. This is only the second time that ketoconazole has been used during pregnancy for the treatment of Cushing's syndrome. We argue that ketoconazole may be safe as well as effective in pregnancy and, furthermore, without any consequences for the child.- - - - - - - - - - ranking = 1keywords = pregnancy, gestation (Clic here for more details about this article) |
2/51. pheochromocytoma and sub-clinical Cushing's syndrome during pregnancy: diagnosis, medical pre-treatment and cure by laparoscopic unilateral adrenalectomy.The coexistence of pheochromocytoma and primary adrenal Cushing's syndrome of the same adrenal gland has rarely been reported. We describe here the case of a female patient presenting with mild Cushing's stigmata, hypertension and diabetes mellitus in whom we diagnosed a pheochromocytoma of the left adrenal gland with coexisting non-ACTH-dependent cortisol hypersecretion. While hormonal work-up was still in progress, the patient became pregnant and wanted to carry her pregnancy to full-term. A laparoscopic adrenalectomy in the 17th week of gestation was decided upon and the patient accordingly prepared for surgery by pre-treatment with phenoxybenzamine. Successful surgery--the first ever reported laparoscopic resection of a pheochromocytoma in pregnancy--without perioperative complications was performed under general anesthesia, with the patient receiving peri- and post-operative hydrocortisone substitution. Pathohistological examination revealed a pheochromocytoma with positive immunostaining for interleukin-6 (IL-6) and negative immunostaining for ACTH, vasoactive intestinal polypeptide (VIP) and cytochrome P450, and with no signs of malignancy. A paracrine stimulation of the ipsilateral adrenal cortex by IL-6 produced by the pheochromocytoma, leading to cortical hyperplasia and subclinical Cushing's syndrome, is suggested by the positive immunostaining for IL-6 and the MRI findings. Post-operatively, secondary adrenal insufficiency ensued, necessitating continuing hydrocortisone replacement over 12 months. hypertension resolved after surgery, and diabetes after the uncomplicated vaginal delivery at term.- - - - - - - - - - ranking = 0.67698824859343keywords = pregnancy, gestation (Clic here for more details about this article) |
3/51. Recurrent ACTH-independent Cushing's syndrome in multiple pregnancies and its treatment with metyrapone.A 17-year-old primigravid woman presented with Cushing's syndrome. Typical clinical symptoms and signs developed at the beginning of pregnancy. By week 17 of gestation, plasma cortisol diurnal rhythm was absent and there was a paradoxical increase in plasma cortisol after a 1-mg dexamethasone overnight suppression test. Basal urinary free cortisol was 10 times above the upper limit (in pregnancy) and ACTH levels were suppressed. The diagnosis of ACTH--independent Cushing's syndrome was established. MRI scans revealed normal adrenal and pituitary glands. To control hypercortisolism, the patient was treated with metyrapone. At 34 weeks of gestation, the patient developed preeclampsia and underwent caesarean section. A female infant weighing 1070 g was delivered. No apparent metyrapone-induced teratogenic effects were observed. Cushing's syndrome in the patient resolved within three weeks of delivery. No corticosteroid replacement therapy either for child or mother was needed. Eight months after delivery the patient became pregnant again and rapidly developed Cushing's syndrome with typical clinical symptoms and signs and laboratory results (urinary free cortisol 6464 nmol/24 h). This second pregnancy was unwanted and terminated by artificial abortion that was followed by rapid resolution of hypercortisolism. A third pregnancy, 12 months after delivery was also accompanied by the rapid development of hypercortisolism which recovered after artificial termination. The mechanisms by which pregnancy-induced Cushing's syndrome occurred in this patient are unclear. Aberrant responsiveness or hyperresponsiveness of adrenocortical cells to a non-ACTH and non-CRH substance produced in excess in pregnancy should be considered. metyrapone suppression of hypercortisolism currently represents the best treatment for these rare cases.- - - - - - - - - - ranking = 0.70795299437371keywords = pregnancy, gestation (Clic here for more details about this article) |
4/51. carney complex--an unexpected finding during puerperium.carney complex is an extremely rare, autosomal dominant, multi-system disorder characterized by multiple neoplasias and lentiginosis. The genetic defect responsible for this complex has been localized to the short arm of chromosome 2 (2p16). The most prevalent clinical manifestations in patients with carney complex are spotty skin pigmentation, skin and cardiac myxomas, Cushing's syndrome and acromegaly. Here we report the case of a 31-year-old woman with a spontaneous pregnancy. At 32 weeks of gestation, she was admitted to our Department of obstetrics with hypertension and severe back pain. In addition, she had unusual pigmentation and typical cushingoid features. One day after admission, the pregnancy was terminated by emergency cesarian section because of preeclampsia and pathological CTG. During the postoperative period the severe back pain persisted, and radiographic evaluation revealed a collapse of L(2)/L(3) with severe osteopenia. A CT scan showed a mass in the right suprarenal area. Histopathological examination revealed a primary pigmented nodular adrenocortical disease. After biochemical confirmation of the diagnosis of Cushing's syndrome, it was recognized that the patient met the diagnostic criteria for carney complex.- - - - - - - - - - ranking = 0.24630591338467keywords = pregnancy, gestation (Clic here for more details about this article) |
5/51. Cushing's syndrome secondary to adrenal adenoma during pregnancy.Cushing's syndrome during pregnancy is rare because of the suppressive effect of excessive glucocorticoid on the female reproductive system. Adrenal adenoma is the most common cause of Cushing's syndrome during pregnancy. Surgical treatment by unilateral adrenalectomy was preferred during pregnancy before the advent of laparoscopic adrenalectomy. We describe two patients with Cushing's syndrome secondary to adrenal adenoma diagnosed during the first and second trimester, respectively, and successfully managed by different approaches of laparoscopic adrenalectomy--one patient upon diagnosis and one after pregnancy.- - - - - - - - - - ranking = 0.86136467041753keywords = pregnancy (Clic here for more details about this article) |
6/51. Case report of a Cushing's syndrome patient with multiple pathologic fractures during pregnancy.We describe a case of multiple pathologic fractures in a woman with Cushing's syndrome during pregnancy. Pathologic fractures were seen in the pubic and ischial bones and in T12 and L1 of the vertebral body. These findings, accompanied by easy fatigue, amenorrhea without nursing, psychological disorder, and premature birth, were retrospectively compatible with those of Cushing's syndrome, not pregnancy-associated osteoporosis. After adrenalectomy, the pain in her groin and back ceased. Plain radiographs showed healing of the fractures and increased mineralization. This case demonstrates the need to be alert to the possible presence of metabolic bone disease, including Cushing's syndrome, when we encounter multiple pathologic fractures with osteoporosis in young patients, even if the patient is pregnant.- - - - - - - - - - ranking = 0.64602350281314keywords = pregnancy (Clic here for more details about this article) |
7/51. Cushing's syndrome during pregnancy: curative adrenalectomy at 31 weeks gestation.A case of Cushing's syndrome due to benign adrenal adenoma (Ad) arising in pregnancy is described. Accurate tumour localisation with magnetic resonance imaging facilitated definitive surgical intervention. Curative adrenalectomy was performed via a posterior approach in the third trimester with subsequent uncomplicated delivery of a healthy infant.- - - - - - - - - - ranking = 0.66221190213209keywords = pregnancy, gestation (Clic here for more details about this article) |
8/51. pregnancy-associated Cushing's syndrome secondary to a luteinizing hormone/human chorionic gonadotropin receptor-positive adrenal carcinoma.Cushing's syndrome occurring during pregnancy is frequently due to an adrenal neoplasm. Adrenal gland tumors occasionally respond to luteinizing hormone (LH) or human chorionic gonadotropin (hCG). We report a case of Cushing's syndrome during and following pregnancy due to an adrenal carcinoma which expressed the LH/hCG receptor. The presence of these receptors may have led to the growth and function of the tumor during pregnancy.- - - - - - - - - - ranking = 0.32301175140657keywords = pregnancy (Clic here for more details about this article) |
9/51. A case of Cushing s syndrome in pregnancy secondary to an adrenal cortical adenoma.Cushing s syndrome in pregnant women is rare and difficult to be diagnosed because of the syndrome's association with oligomenorrhea or amenorrhea and the changes in cortisol metabolism during normal pregnancy. cushing syndrome in pregnancy is usually confused with complicated pregnancy, such as preeclampsia or gestational diabetes, and its rarity leads to a low degree of clinical suspicion, often delaying diagnosis. We experienced a case of Cushing s syndrome in pregnancy, which had been considered as the severe preeclampsia and gestational diabetes due to uncontrolled hypertension and hyperglycemia. The pregnancy was terminated with an emergency cesarean operation at 30 weeks of gestation because of severe preeclampsia. In consequence of the evaluation about the Cushing s syndrome after delivery, the adrenal cortical adenoma of right adrenal gland was diagnosed and laparoscopic adrenalectomy was performed.- - - - - - - - - - ranking = 1.0619294915606keywords = pregnancy, gestation (Clic here for more details about this article) |
10/51. A case of ACTH-producing pheochromocytoma associated with pregnancy.Ectopic ACTH syndrome is rarely caused by pheochromocytoma. We report a case of a 28-year-old woman with Cushing's syndrome due to ACTH-producing adrenal pheochromocytoma. She had delivered preterm baby at 32nd week of gestation with 'severe preeclampsia'. After delivery, persistent hypertension accompanied by severe headache led her to being misdiagnosed as Cushing's syndrome due to right adrenal adenoma (normal plasma ACTH level) and cerebral vasculitis of unknown etiology. She was referred to our hospital for surgical treatment. Repeated biochemical studies suggested coexistence of ectopic ACTH syndrome and pheochromocytoma. To reverse her clinical deterioration, right total and left subtotal adrenalectomy was performed with presumptive diagnosis of 1) right adrenal pheochromocytoma causing ectopic ACTH syndrome or 2) coexistence of ACTH-dependent Cushing's syndrome and right adrenal pheochromocytoma. Pathologic examination of right adrenal mass revealed pheochromocytoma which showed strong immunostaining for ACTH. plasma ACTH and urinary cortisol excretion normalized after surgery, but she succumbed to multiple cerebral infarcts and disseminated intravascular coagulation. pregnancy and inappropriately low plasma ACTH at initial evaluation might have hampered early diagnosis. To our knowledge, this is the first description of a case with ectopic ACTH syndrome due to pheochromocytoma associated with pregnancy.- - - - - - - - - - ranking = 0.56931766479124keywords = pregnancy, gestation (Clic here for more details about this article) |
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