A thyroid nodule revealing a paraganglioma in a patient with a new germline mutation in the succinate dehydrogenase B gene.

A 32-year-old asymptomatic female was diagnosed with an isolated thyroid nodule of 2.5 cm diameter. Fine needle aspiration suggested a medullary thyroid carcinoma.

[Post-radiotherapy hyperthyroidism: a rare complication of cancer treatment in the child].

Unlabelled: Abnormalities of thyroid function, specially hypothyroidism, are common complications of head and neck irradiation for childhood cancer. Hyperthyroidism is rare and can be misdiagnosed. We report two observations of this condition.

[Cushing's syndrome and adrenal insufficiency in pregnancy].

During pregnancy, major changes of the corticotroph axis activity are observed. The placenta synthetizes Corticotropin-Releasing Hormone (CRH) and pro-opio-melanocortin (POMC), and the plasma levels of both peptides are highly increased during pregnancy. The cortisol plasma levels are two-fold elevated compared to the levels observed in non pregnant women.

[Magnetic resonance imaging in Cushing disease. Prediction of surgical results].

Objectives: The purpose of the study is to determine if pituitary Magnetic Resonance Imaging (MRI) can predict the outcome of transsphenoidal surgery in patients with Cushing's disease.

Methods: Fifty four patients were divided in three groups according to MRI findings: those with a well circumscribed focal lesion clearly separated from the cavernous sinus (group 1, n = 24), those with adenomas in close contact with the cavernous sinus (group 2, n = 18), and those with no identified lesion (group 3, n = 12).

Results: The adenoma is found on the predicted side in 97,6% of the cases with positive MRI.

Comparison of computerized tomography and magnetic resonance imaging for the examination of the pituitary gland in patients with Cushing's disease.

Objective: The vast majority of patients with Cushing's disease have a corticotroph adenoma, the selective removal of which, through the transsphenoidal route, has the potential to offer a definitive and complete cure. This study was designed to compare the diagnostic accuracy of computerized tomography (CT) and magnetic resonance imaging (MRI) to identify the presence, evaluate the size, and assess the topographic characteristics of pituitary corticotroph adenomas.

Methods: Forty-two patients with Cushing's disease were included in this prospective study, of whom 16 were subsequently explored transsphenoidally.

[Prolactin adenoma developed during puberty. Diagnosis and long-term course. 9 cases].

Nine patients (2 boys, 7 girls) with prolactinomas diagnosed at puberty were followed for 2 to 20 years. The presenting signs were sexual infantilism (n = 2), amenorrhea-galactorrhea (n = 6) and hypogonadism (n = 1). Growth retardation was also present in 3 cases.

[Cushing syndrome during pregnancy. New diagnostic methods used in 3 cases of adrenal cortex carcinoma].

Cushing's syndrome during pregnancy is most often caused by an adrenal cortical tumour; it is a rare event which bears poor foetal and maternal prognoses. We report 3 cases of adrenal cortex carcinoma diagnosed during pregnancy (after 24, 27 and 28 weeks respectively of amenorrhea) and revealed by local tumoral signs in 2 cases and by pulmonary embolism in the third. Because hair growth was moderate and weight gain as well as high blood pressure had mistakenly been attributed to the pregnant state, these clinical features of hypercortisolism has only lately been related to tumoral secretion.

Cushing's syndrome and pregnancy: aetiologies and prognosis in twenty-two patients.

Objective: The association of an active Cushing's syndrome and pregnancy is a rare event which raises specific diagnostic difficulties and bears poor maternal and foetal prognoses. We report our series of 22 patients.

Patients: Age range was 19-34 (mean +/- SEM = 27.

[Nodular adrenomegalies in congenital adrenal hyperplasia in adults. Implications].

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.

Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy.

Adrenocortical carcinoma is a rare tumor, and only limited information is available about its natural history and the effects of therapy. We studied 105 patients (75 female and 30 male; mean age, 46 years) with adrenocortical carcinoma who were referred to us between 1963 and 1987. The average duration of symptoms before diagnosis was 8.

[Short term effects of ketoconazole in Cushing's syndrome].

The effects of Ketoconazole (600 mg/day) were evaluated in 10 patients with Cushing's syndrome during a mean period of 4.5 weeks (range 1-12). The urinary free cortisol excretion (UFC) decreased by 21 +/- 15% (mean +/- SEM) (p less than 0.

Transsphenoidal pituitary surgery for the treatment of Cushing's disease: results in 64 patients and long term follow-up studies.

Transsphenoidal pituitary surgery was performed in 64 patients with Cushing's disease in search of a corticotroph adenoma. In 4 patients, profuse local bleeding prevented completion of the exploration. Of the 60 patients who had an adequate exploration, 4 could not be followed after surgery.

Salivary cortisol measurement: a practical approach to assess pituitary-adrenal function.

The salivary cortisol concentration is an excellent indicator of the plasma free cortisol concentration. To establish its normal and pathological ranges, salivary cortisol concentrations were measured in 101 normal adults, 18 patients with Cushing's syndrome, and 21 patients with adrenal insufficiency. The normal subjects had a mean (+/- SEM) salivary cortisol concentration of 15.

[Adrenomegaly and other masses of the adrenal area. Diagnostic and therapeutic approaches].

During the 1982-1987 period 104 patients were surgically explored for a mass of the supra renal area. An adrenal tumor was found in 67%, an adrenal pseudo-tumor in 12.5%, a non adrenal pathology in 12.

[Tumor and pseudotumor lesions of the adrenal area not to be misdiagnosed].

Thirteen patients with a mass in the adrenal gland area discovered at ultrasonography or computed tomography were studied. Hormone levels were normal in all but three patients with adrenal insufficiency. With the exception of three patients with metastatic tumours or adrenal lymphoma, all were operated upon on account of complications or for diagnostic purposes.

[The hormonal state of pregnancy: modification of cortisol and testosterone].

The pattern of cortisol and testosterone levels during the normal pregnancy was investigated by measuring these hormones in the same 19 healthy pregnant women at 11th to 19th, 24th to 29th and 34th to 39th week post amenorrhea. We noted the well-known increase in total plasma cortisol and testosterone, due to the elevated concentration of their transport protein, i.e.

[Rapid beta 1-24-corticotropin test in the exploration of the corticotropic axis. 71 cases].

The reliability of the beta 1-24 corticotropin test, which is both cheap and safe, in the assessment of the hypothalamic-pituitary-adrenocortical function was re-evaluated by comparing its results with those of the metyrapone test in 71 subjects: 30 controls and 51 patients with suspected pituitary-adrenal dysfunction. Response to a 500 micrograms intramuscular injection of beta 1-24 corticotropin was evaluated by measuring the 60 min plasma cortisol levels; 4.5 g of metyrapone divided into 6 doses were then administered orally and response was evaluated by measuring plasma 11-desoxycortisol levels.

[Paraneoplastic hypercorticism].

Paraneoplasic hypercorticism results from ectopic corticolipotropic secretion. Its incidence remains underestimated because of the latent clinical forms of Cushing's disease. In some cases the primary tumour (bronchopulmonary, thymic, pancreatic or other) presents late.

[Cushing's disease. Current data on its course and treatment].

Recent developments in the treatment of Cushing's disease, particularly the trans-sphenoidal microadenomectomy, have prompted a survey of the pathophysiological theories of ACTH hypersecretion in this condition. The results of the trans-sphenoidal approach are reported and the post-surgical evolution (success failure or recurrence) are discussed. It is emphasized that a long fellow up is essential to assess the efficacy of this therapeutic approach.

[Results of surgical treatment of Basedow's disease].

Diffuse hyperthyroidism occurred in 88 patients (82 women and 6 men), aged 14 to 74 years, and was due to Graves disease in 65 cases and heteromultinodular goiter in 23 cases. After medical preparation, the surgical procedure was subtotal thyroidectomy for Graves disease and total unilateral with partial controlateral lobectomy for heteromultinodular goiter. The immediate postoperative course was uneventful except for two transient acute episodes of thyrotoxicosis.