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The authors report the hormonologic characteristics of 20 obese and hirsute women meeting the criteria for adrenaltype hyperandrogenism, suppressible by dexamethasone, without hyperprolactinemia and without any late developing partial enzyme block appearance. The laboratory profile of these women differed from that of a group of women with type 1 polycystic ovaries syndrome. In this same group obese women in whom LH/FSH ratio was below 1, there was evidence under baseline conditions of a moderate increase in testosterone and delta 4-androstenedione in relation to increased plasma levels of DHA and SDHA, plasma delta 4 and delta 5-androgen levels falling precipitalely during the dexamethasone suppression test. The ACTH stimulation test revealed greater reactivity for 17 hydroxy-pregnenolone (p < 0.001) and less for 21-deoxycortisol than in the control group of normal women (p < 0.01). The essentially adrenal origin of plasma hyperandrogenism in certain cases of obesity is discussed. Insulin could increase adrenal sensitivity to ACTH and its possible action in vivo on the activity of adrenal enzymes requires clarification. The accumulation of certain androgens in the adrenal cortex could also be responsible for dysregulation of 3 beta ol-dehydrogenase and 11-hydroxylase.

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OBJECTIVES: Nodular or pseudo-nodular aspects of Hashimoto's thyroiditis raise the problem of the association with a differentiated carcinoma or a non Hodgkin's lymphoma. METHODS: We looked for patients needing surgery in 165 cases of Hashimoto's thyroiditis. For this purpose, we used fine needle aspiration cytology. RESULTS: We found a differentiated carcinoma in 4% of cases and a non Hodgkin's lymphoma in 1% of cases. CONCLUSION: In nodular or pseudo-nodular aspects of Hashimoto's thyroiditis, fine needle aspiration cytology is helpful for the nodule diagnosis and for the selection of suspicious nodules only to be referred to surgery.

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Recent advances in molecular genetics now enable the study in vitro of follicular maturation using human recombinants of gonadostimulins (FSH and LH) and certain autocrine and paracrine ovarian regulation factors. This is leading to reevaluation of the precise role played by the various hormonal protagonists of folliculogenesis. FSH, in the presence of low levels of LH at the start of the follicular phase, has a mitotic action on estrogen-producing granulosa cells, encourages their differentiation and activates the synthesis of their genetically-determined products (essentially somatomedin, inhibin and activin) and of the LH receptor. High LH levels, during the second half of the follicular phase, stop proliferation of granulosa cells, and, synergistically with insulin-like growth factor 1, bring about an increase in estrogen synthesis from aromatisable androgens. In common with FSH, LH facilitates the production of cybernins and of essential growth factors, as well as of preovulatory progesterone. Abnormalities of endocrine, paracrine or autocrine regulation of follicular maturation may lead to atresia. Future rational uses of human FSH and LH are obvious and involve the optimisation of ovulation induction and in vitro fertilisation in sterile women.

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The detection of clinical hyperandrogenism in women presenting with infertility requires detailed hormonal investigations using the decisional plan suggested here. Initial studies including measurement of plasma androgen, gonadotrophic hormones and prolactin levels, may be sufficient to reveal an adrenal origin or pure ovarian origin. Non-tumor androgenic hypercorticism is seen classically in late-presenting enzyme deficits, but also in other situations: excessive adrenarche, hyperprolactinemia, obesity, chronic stress. The immediate Synacthene test can then eliminate diagnostic uncertainties if it leads to the discovery of appearances of 21- or 11-hydroxylase or 3 beta-ol dehydrogenase blocks. Intense virilisation in a woman with a testosterone level above 2 ng/ml (7 nM/l) should lead to suspicion of an androgen-secreting tumor of the ovary or adrenal. CT scan of the abdomen and true pelvis is essential here since it may reveal the presence of an adrenal or ovarian mass. If no morphological abnormality is shown by this investigation, an endocrine lesion of a small ovary should be strongly suspected, the demonstration of which requires isotope techniques and/or catheterisation of the ovarian veins. Two situations also exist which are responsible for severe hyperandrogenism but less alarming in terms of their course and significance: certain homozygous forms of 21-hydroxylase deficit diagnosed late and ovarian hyperthecosis. It may happen that these hormonal investigations do not suffice alone to determine the precise origin of hyperandrogenism and its cause. The dexamethasone adrenal suppression test is useful in the diagnosis of type II micropolycystic dystrophy, in order to define the essentially ovarian, adrenal or mixed origin of hyperandrogenism.

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This study reviews the various types of treatment used in infertility due to hyperandrogenism, with the aim of answering the following three questions: How should these drugs be prescribed? What are their side-effects? What are the best diagnostic indications? Possibilities include ovulation inducers but also all types of treatment capable of improving the fertility of these women. The treatment of infertility due to adrenal hyperandrogenism is based upon glucocorticoids. This treatment must be continued for 3 months after conception, to attempt to avoid early spontaneous abortion. The first-line inducer in ovarian hyperandrogenism is clomiphene citrate. The good results obtained using the combination of dexamethasone and clomiphene citrate are explained by an adrenal participation in this type of hyperandrogenism. In case of failure, and in addition to classical menotrophins:--pre-treatment using LHRH agonists avoids the onset of premature luteinisation but does not prevent the possibility of multiple pregnancies;--use of purified FSH reduces, though not sufficiently, the risks of multifollicular maturation but does not greatly increase the overall pregnancy rate;--the "slow" protocol with purified FSH reduces the incidence of multifollicular maturation. Should this fail, prior treatment with an LHRH agonist and if not the pulsed administration of LHRH in non-obese women can be suggested. Surgical treatment provides useful results in severe forms of sterility due to polycystic ovaries syndrome, with new per-celioscopic techniques.

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The clinical and therapeutic aspects of fertility due to hyperandrogenism were studied in 49 women (40 presenting with sterility and 9 for another reason but seeking to become pregnant). Patients were divided into three main etiological groups on the basis of hormonal findings: ovarian (15 cases), adrenal (16 cases) and mixed (11 cases) hyperandrogenism. This study highlighted several points. 22% of infertile women had no cutaneous signs of hyperandrogenism and menstrual disturbances were missing in 46% of cases of adrenal hyperandrogenism. Primary infertility was seen more often in all types of hyperandrogenism (28 cases) than secondary infertility (12 cases). Secondary infertility was explained by a high early spontaneous abortion rate in hyperandrogenism (40%). This was much commoner in adrenal hyperandrogenism than in ovarian or mixed hyperandrogenism. Inducing treatment based upon a combination of dexamethasone and clomiphene citrate proved to be the most effective in these infertile women (86% pregnancy rate). The frequency of spontaneous abortions in infertility due to ovarian or mixed hyperandrogenism treated by clomiphene citrate alone can probably be explained by the persistence of hyperandrogenism.

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Abetalipoproteinemia is a recessive genetic disorder of unknown origin, which is characterized by absence of circulating apo-B-containing lipoproteins, malabsorption of intestinal fat, and degenerative neurological and retinal lesions. In this study, four families were analysed for genetic linkage between the abetalipoproteinemia phenotype and the apo-B genotype determined from polymorphisms of XbaI, MsPI, EcoRI and PvuII restriction sites and that of the 3'-minisatellite of the apo-B gene. The results definitively exclude mutation of the apo-B gene as a causal factor of abetalipoproteinemia in three families. Consanguinity of the parents in the fourth family made genotyping less conclusive.

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Late-onset congenital adrenal hyperplasia is a cause of a spectrum of clinical manifestations of postnatal androgen excess. In these cases, ACTH stimulation test with measurement of 17-Hydroxyprogesterone (17OHP) is usually done to assess 21-hydroxylase (21-OH) deficiency. Determination of the 11-deoxycortisol (S) and the S to cortisol ratio is rarely done, so that 11 beta-hydroxylase (11-OH) deficiency seems unusual. We systematically investigated this biosynthetic defect among women complaining of hyperandrogenism (n = 519) and, comparing the patient's hormonal responses to ACTH with those of 31 normal women, found 29 11-OH deficiency (5.6%): this is the largest group ever reported. S was elevated only 9 times, so that using this single determination, diagnosis of 20 enzymatic defects would not have been made. Only three of the patients (10%) had hypertension, even though the pathway of aldosterone was involved in 33% of cases (criteria: elevation of the ratio desoxycorticosterone to corticosterone). We also described one new patient with both 11-OH and 3-beta-hydroxysteroid dehydrogenase deficiencies. The patho-physiology is particularly interesting in these cases. It is concluded that the single research for 21-OH deficiency is inadequate among women complaining of hyperandrogenism: the screening for 11-OH deficiency should be made, even if blood pressure is normal.

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