[Manifestations and prognosis of thyrotropin-secreting pituitary adenomas: a case series of three patients].

Introduction: Among pituitary adenomas, thyrotropinomas were previously considered as extremely rare and resistant to therapy. However, the common use of the sensitive TSH measurement and the improvement of pituitary imaging have modified their clinical and hormonal presentation.

Case Reports: We here report three cases of TSH secreting pituitary adenoma that highlight the great diversity of the clinical, hormonal and morphological presentation, and their better prognosis.

Adrenocortical carcinoma and pregnancy: clinical and biological features and prognosis.

Objective: Adrenocortical carcinoma (ACC) is a rare, severe disease. Pregnancy-associated ACC has rarely been reported. We wished to evaluate the characteristics and prognosis of ACC diagnosed in patients during pregnancy or in the postpartum period, comparing them with those for ACC diagnosed in nonpregnant women.

Clinical and molecular characterization of a cohort of 161 unrelated women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency and 330 family members.

Context: Nonclassical congenital adrenal hyperplasia (NC-CAH) due to partial 21-hydroxylase deficiency is one of the most frequent autosomal recessive diseases.

Objective: The aim of this study was to determine the genotype/phenotype relationship in probands and family members.

Patients And Methods: A total of 161 NC-CAH unrelated women diagnosed on late-onset symptoms, mainly hirsutism, and post-ACTH 17-hydroxyprogesterone more than 10 ng/ml, and 330 of their relatives was explored.

Luteinizing hormone pulsatility in patients with major ovarian hyperandrogenism.

Hyperandrogenism and ovulatory dysfunction are common in women with either polycystic ovary (PCOS) or ovarian virilizing tumor. However, contrasting with the numerous studies that have extensively described gonadotropin secretory abnormalities, principally increased LH pulse amplitude and frequency, few studies have concerned gonadotropin secretion in patients with ovarian virilizing tumors; low gonadotropin levels have occasionally been reported, but never extensively studied. The goal of the present study was to further evaluate the pulsatility of LH secretion in women with ovarian virilizing tumor compared with that of PCOS patients.

[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.

Phenotype-genotype correlation in 56 women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Complete analysis of the CYP21 gene was performed in 56 unrelated French women with symptomatic nonclassical congenital adrenal hyperplasia. The mutational spectrum and the phenotype-genotype correlation were examined. The overall predominant mutation was V281L, which was present on 51% of alleles and in 80% of women.

[Diabetes insipidus with a hypothalamo-hypophyseal morphologic anomaly during a pregnancy].

Background: Diabetes insipidus is uncommon in pregnancy. Despite physiological modifications in hydroelectrolytic balance during normal pregnancy, the capacity of the kidney to concentrate urine is preserved, partially due to lower vasopressin secretion.

Case Report: A young woman developed diabetes insipidus during the third trimester of normal pregnancy.

[Differentiated thyroid carcinomas manifesting as toxic adenoma].

Background: Hot thyroid nodules are generally benign. We report two exceptional cases of thyroid carcinomas mimicking toxic adenomas.

Case Reports: A 35-year-old man and a 55-year-old woman had thyroid carcinoma behaving as an autonomously hyperfunctioning nodule.

[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.

Fertility in women with late-onset adrenal hyperplasia due to 21-hydroxylase deficiency.

Fertility was evaluated in 53 female patients with late-onset adrenal hyperplasia (LAH) due to 21-hydroxylase deficiency. The majority of patients (n = 33) were seen for isolated postpubertal hirsutism, 9 patients consulted for sterility, and 11 for irregular menstrual cycles. At the time of diagnosis, the ages of patients ranged from 15-40 yr (mean +/- SD, 24.

[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.

Cyproterone acetate versus hydrocortisone treatment in late-onset adrenal hyperplasia.

Thirty late-onset adrenal hyperplasia patients consulting for isolated hirsutism were randomly divided into two groups; group 1 (n = 16) was treated with hydrocortisone in order to suppress androgen adrenal secretion, and group 2 (n = 14) received cyproterone acetate (CPA) antiandrogen therapy to inhibit peripheral androgen activity. The clinical and hormonal effects of each type of treatment were evaluated. Before treatment, the clinical and hormonal profiles of the two patient groups did not differ significantly.

[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.

Evening urinary free corticoids: a screening test in Cushing's syndrome and incidentally discovered adrenal tumours.

In this study, we investigated the usefulness of the determination of evening urinary free corticoids/creatinine in samples collected from 20.00 to 24.00 h as a screening test in Cushing's syndrome.

[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.

Evaluation of diagnostic criteria for Leydig cell tumours in adult men revealed by gynaecomastia.

Gynaecomastia caused by Leydig cell tumours (LCT) in adult men may appear a long time before clinical evidence of testicular swelling. To evaluate the diagnostic criteria for LCT, hormonal status was studied in 14 cases and compared with results of a control group (CG) and 10 men with idiopathic gynaecomastia (IG). The mean plasma T level was significantly (P less than 0.

Late-onset adrenal hyperplasia in hirsutism.

We studied the incidence of late-onset adrenal hyperplasia as a cause of hirsutism, its association with the major histocompatibility complex, and its clinical expression. Twenty-four of 400 women seen because of hirsutism were found to have late-onset adrenal hyperplasia, diagnosed on the basis of a high plasma level of 17-hydroxyprogesterone, and its marked increase after ACTH stimulation. The degree of hirsutism varied widely.

Long-term study of mortality and vascular complications in juvenile-onset (type I) diabetes.

A cohort of 372 insulin-dependent diabetic children, diagnosed between October 1949 and December 1960, were followed-up until December 1976 by the same team of physicians. At the time of diagnosis all patients were under 16 yr of age and were given standardized treatment which did not change from 1949 to 1976. The therapy consisted of daily insulin adjustment based on clinical assessment, the degree of physical activity, and the results of semi-quantitative urine tests for sugar and ketone bodies.