[Antiphospholipid syndrome. A new cause of bilateral hemorrhage of the adrenal glands. 4 cases]. / Syndrome des antiphospholipides. Une nouvelle cause d'hémorragie bilatérale des surrénales. 4 observations.

Four cases of adrenal insufficiency due to bilateral adrenal haemorrhage in patients with antiphospholipid syndrome are reported. The 1st patient had repeated episodes of thrombosis on a background of altered general condition; he was examined by computed tomography (CT) which showed enlarged and presumably tumoral adrenal glands; adrenal insufficiency was present and improved under hormone replacement therapy; the thrombotic episodes were attributed to the antiphospholipid antibodies; after a 5-year follow-up the antiphospholipid syndrome remained alone, and further examinations showed progressive adrenal atrophy. The 2nd patient had systemic lupus erythematosus with thrombocytopenia; because of abdominal pain CT was performed, showing bilateral adrenal enlargement; treatment with intravenous pulses of cyclophosphamide and high-dose immunoglobulins combined with corticosteroids failed, and splenectomy was performed disclosing an old adrenal haematoma which was evacuated. The 3rd patient had bilateral and asymmetrical adrenal hypertrophy at CT; subsequently, systemic lupus erythematosus was diagnosed with anti-prothrombinase and anticardiolipin accounting for the initial findings; follow-up examinations showed the formation of pseudocysts in the adrenals; following myocardial infarction the patient died of cerebral haemorrhage, and autopsy confirmed the presence of old, bilateral adrenal haematomas. The 4th patient had recurrent vein thrombosis associated with distal ischaemia, which prompted CT in search of a neoplasia; this examination revealed 2 large adrenal haematomas while anticardiolipin antibodies were found. In patients with antiphospholipid syndrome any functional or morphological abnormality of the adrenals should prompt a search for bilateral adrenal haemorrhage. Conversely, in all cases of adrenal insufficiency a search for antiphospholipid antibodies should be part of all aetiological investigations, and this search should be carried out prior to withdrawing corticosteroids in cases of systemic lupus erythematosus with antiphospholipid antibodies.

[Acquired corticotropin insufficiency in adults. 2 new cases]. / Insuffisance corticotrope acquise de l'adulte. Deux nouveaux cas.

We report two new cases of isolated corticotropin deficiency, associated with TSH deficiency in one of the two patient. The diagnostic was made difficult because the symptoms were mainly psychiatric. Low plasmatic cortisol and ACTH level were found, with low T3, T4 and TSH in the second patient. The TSH did not respond to the stimulation by TRH. The cerebral tomodensitometry and magnetic resonance imaging focused on the sella turcica were normal, eliminated a tumor and showed an empty sella turcica. The patients have to be followed-up because an isolated deficiency may further complete to panhypopituitarism.

[Intolerance to carbohydrates: the seven questions]. / Intolérance aux hydrates de carbone: les sept questions.

The borderline between diabetes and intolerance to carbohydrates has been drawn on the basis of prospective studies which determined a glycaemic threshold marking the risk for microangiopathy. On the other hand, the borderline between intolerance to carbohydrates and normal glucose tolerance remains arbitrary: 25% for subjects who are intolerant to carbohydrates return to normal glucose tolerance within 10 years. This is due to the fact that intolerance to carbohydrates is a heterogeneous entity which should be dismembered according to the severity of insulin deficiency and to the degree of insulin resistance. Alteration of insulin secretion is perhaps the most specific marker of susceptibility to non insulin dependent diabetes, but insulin resistance is certainly the principal factor exhausting insulin secretion and leading to non insulin dependent diabetes. Insulin resistance and the hyperinsulinism it creates seem to facilitate atherogenesis, even when glucose tolerance is still normal, so that the oral glucose tolerance test is not only poorly reproducible but loses a great deal of its value in the early detection of vascular risk. Measurements of fasting and post-prandial glucose levels and of A1C haemoglobin, cholesterol, triglyceride, and HDL cholesterol levels usually make it possible to classify subjects into one of the three following categories: (1) no risk of macro- or microangiopathy; (2) diabetes with a risk of macro- or microangiopathy; (3) intolerance to glucose with risk of atherogenesis but no risk of microangiopathy. The oral glucose tolerance test probably remains useful within a small set of values that are either very slightly above normal or dissociated. Measuring blood insulin levels might be a better way of assessing the risk of atherogenesis, but the clinical use of this test requires evaluation.

Spectroscopic MRI: a tool for the evaluation of systemic lipid storage disease.

Spectroscopic MR imaging allows to measure the lipid content of a region inside the human body. This technique has been applied to the case of a woman with a severe multisystemic triglyceride storage disease. Lipid contents of liver, pancreas, kidneys, left ventricle, skeletal muscles (calves, thighs, arms) were measured by using the Dixon spectroscopic imaging sequence. In some heterogeneous muscles, localized proton spectra were recorded. Results agreed with clinical findings (muscle weakness, normal renal and cardiac function, diabetes). These techniques could help to quantify the severity of the disease and to follow up its evolution under therapy.

[Lipoprotein Lp(a): a new risk factor of atherogenesis]. / La lipoprotéine Lp (a): nouveau facteur de risque athérogène.

Lp(a) is a lipoprotein present in all individuals in concentrations that are genetically determined. Its structure is characterized by the presence of an apoprotein with a high carbohydrate content called apoprotein a. Since 1972, numerous concordant data have endowed Lp(a) with a high risk of atherogenesis. This risk applies to the coronary and cervico-encephalic arteries. For the latter, Lp(a) even is a lipid parameter regarded as a major risk factor. The origin and metabolism of Lp(a) are little known, by they seem to differ from those of low-density lipoproteins. Its specific apoprotein of Lp(a). At the moment, there is no simple dietetic or medicinal treatment that can lower substantially the serum level of Lp(a).

Complete iodide trapping defect in two cases with congenital hypothyroidism: adaptation of thyroid to huge iodide supplementation.

Two cases of congenital defect in iodide trapping mechanism are related. The absence of thyroid and gastric concentration of 99mTcO4 led to the diagnosis. The study of saliva and gastric:serum concentration ratios confirmed the complete defect. The kinetics of radioiodine studied by external detection showed an early simultaneous decay in the thyroid, the stomach and the left ventricle. Thyroid accumulation of 131I, demonstrated by camera imaging, was estimated to be 0.1% at 48 h. It probably originated from simple diffusion. Iodide supplementation was progressively increased to 4.5 g and 10 g day-1 respectively. It resulted in a normalization of all parameters. Huge doses of iodide did not result in any evidence of hyperthyroidism as TSH rose normally after TRH. Intermittent iodide supplementation in one case could not maintain euthyroidism longer than a few weeks. Daily treatment, therefore, seems necessary.

[Value of hormonal markers in the detection and therapeutic surveillance of malignant adrenal cortical carcinoma]. / Intérêt des marqueurs hormonaux dans le dépistage et la surveillance thérapeutique des corticosurrénalomes malins.

The value of hormonal markers in the diagnosis and early and late surveillance of adrenocortical carcinoma was assessed in a series of 10 cases and from a review of the literature. Although hormonal investigation with measurement of precursors, performed whenever there is the least clinical suspicion of ACC, is of certain diagnostic value, the possibilities of postoperative controls have not yet been fully exploited: the results are difficult to interpret because of possible interference due to the secretion of the controlateral adrenal; plasma renin activity is rarely assessed in the late postoperative period. A general strategy of investigation and surveillance is proposed.

[Hypertension in malignant tumors of the adrenal cortex. A review of clinical, biological and hormonal features]. / L'hypertension artérielle dans les corticosurrénalomes malins. Révision de son expression clinique, biologique et hormonale.

Hypertension may be a prominent and sometimes the dominant expression of tumours of the adrenal cortex as demonstrated in a personal series of 9 cases and in comparable series in the literature. It is therefore important, despite their relatively low incidence, to recognise adrenal cortical tumours as the cause of hypertension. The present study was undertaken to show that this diagnosis is relatively easy. The simplest indicators are:--the change in body electrolytes with hypokalaemia and hyper-kaliuria is practically constant and should prompt a hormonal investigation to demonstrate increases adrenal secretion of mineralocorticoids;--there is also a high incidence, despite the variable profiles of plasma and urinary corticosteroids, of paradoxical lowering of plasma aldosterone in parallel with normal or low PRA which does not react to orthostatism. These two preliminary investigations are the prelude and invitation to the measurement of other mineralocorticoids, especially desoxycorticosterone secondary to a relative block of 11-betahydroxylation from what other in vivo and in vitro studies have shown. Repeated monitoring of the preceding investigations is important for the post-operative follow-up and diagnosis of recurrences or metastases.

[Progressive external ophthalmoplegia and Hashimoto's thyroiditis]. / Ophtalmoplégie externe progressive et thyroïdite de Hashimoto.

Signs of moderately increased metabolism in a patient with progressive external ophthalmoplegia and mitochondrial anomalies on muscle biopsy revealed the presence of Hashimoto's thyroiditis. The relations between ophthalmoplegia plus and thyroid dysfunction are discussed.

Pituitary prolactinoma, adrenal aldosterone-producing adenomas, gastric schwannoma and colonic polyadenomas: a possible variant of multiple endocrine neoplasia (MEN) type I.

A large pituitary prolactinoma was found in a mentally deficient 45 year old woman presenting with amenorrhoea, galactorrhoea, headache, anaemia and hypertension, and removed surgically. She was subsequently found to have multiple adrenal aldosterone-producing adenomas, a gastric schwannoma and colonic polyadenomas. All these tumors were also removed surgically. Despite the absence of parathyroid and islet-cell hyperplasia, this case seems to be a variant of multiple endocrine neoplasia (MEN) type I.

Exaggerated prolactin response of thyrotropin-releasing hormone in women with anovulatory cycles: possible role of endogenous estrogens and effect of bromocriptine.

Twenty-one women 18 to 36 years old, presenting with chronic anovulation, were compared with 10 normally cycling women. The patients were characterized by low progesterone (P) levels (0.93 +/- 0.14 ng/ml versus 15.5 +/- 1.4 in controls), whereas 17 beta-estradiol (E2) was moderately decreased (110.2 +/- 8.3 pg/ml versus 162.8 +/- 14.5 in controls) realizing a relative hyperestrogenism. Basal prolactin (PRL) levels were not elevated (12.1 +/- 0.97 ng/ml versus 9.2 +/- 0.7 in controls), but after thyrotropin-releasing hormone (TRH) stimulation an exaggerated response was observed (114.5 +/- 7 ng/ml versus 55.8 +/- 9 in controls). Patients were treated with bromocriptine (1.25 mg 2 times a day) for 3 months. Fifteen responded with ovulatory cycles, and five became pregnant. Progesterone increased significantly (10.2 +/- 1.3 ng/ml), whereas in patients who did not ovulate P increased only slightly (1.56 +/- 0.18 ng/ml). The particular endocrine profile of these patients (P/E2 imbalance) realizing relative hyperestrogenism may be responsible for the exaggerated PRL response to TRH. Bromocriptine, in reducing this transient, or masked, hyperprolactinemia, allows in many patients the return to ovulatory cycles. This mechanism may be one of the possible pathways leading to chronic functional or organic hyperprolactinemia.

[Inappropriate thyrotropin secretion. Part 1: tumoral (author's transl)]. / Sécrétion inappropriée de thyréostimuline. 1re partie: production tumorale.

Release of thyroxine and triiodothyronine from the thyroid gland is stimulated by the pituitary hormone thyrotropin, or thyroid-stimulating hormone (TSH). TSH secretion in turn is regulated by control mechanisms, which include a negative feedback effect of the thyroid hormones themselves and the actions of the hypothalamic peptide TRH and of several central neurotransmitters. Hyperthyroidism secondary to excessive TSH secretion is a rare entity. In most cases TSH hypersecretion results from an adenoma of the pituitary gland and may then be associated with increased prolactin or growth hormone production. It should be emphasized that most patients with pituitary adenoma have high serum levels of alpha TSH subunit and undetectable beta TSH subunit.