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.

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.

[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.

[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.

[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.

[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.

[Discrepancies between TRH test and classical dynamic thyroid tests results in hyperthyroidism. 47 cases (author's transl)]. / Discordances entre test à la TRH et tests dynamiques classiques dans l'exploration de l'hyperthyroïdie. 47 malades.

We have investigated the frequency of discordant results between the TRH test and classic dynamic tests of thyroid function (thyroid suppression and TSH stimulation tests) for patients presenting a diagnostic problem for hyperthyroidism. 12 patients in group I posed the problem of having a relapse of Graves' disease previously treated: a discrepancy between TRH and T3 suppression test results was observed in 17% of the cases. 10 patients in group II demonstrated the ophtalmopathy of Graves' disease without hyperthyroidism: discordance between tests results in this group was present for 20%. Finally, 25 patients in group III had a "hot" thyroid nodul apparent at thyroid scan: in 40% of the cases, the response to TRH did not lead to the same diagnosis that was suggested by the classical dynamic tests of thyroid regulation. The discrepancies observed in this work, between the results of the TRH test and those of classic dynamic tests, demonstrate that this two types of tests are not completely interchangeable, because they do not reflect exactly the same function.

[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.

[Inappropriate thyrotropin secretion. Part 2: non tumoral (author's transl)]. / Sécrétion inappropriée de thyréostimuline. 2e partie: production non tumorale.

Some patients are clinically euthyroid despite high thyroid hormones levels associated with detectable but not elevated serum TSH. These patients are considered as being resistant to thyroid hormones. The resistance may be severe or partial and in most cases seems to be autosomal dominant; it affects some tissues more than others, thus giving rise to a variety of clinical symptoms. In a few patients with high TSH levels extensive studies have failed to provide evidence of pituitary tumour of resistance to thyroid hormones; the cause of TSH hypersecretion in such cases remains uncertain. Several factors (non specific serum proteins, cross-reactivity circulating antibodies) may result in falsely high T3, T4 and TSH values on radioimmune assays and must be carefully investigated in the presence of unexpectedly high serum TSH levels.

[Dynamic study of the changes in the composition of fatty acids in serum lipid fractions under a diet rich in polyunsaturated fatty acids]. / Etude dynamique de la modification du profil des acides gras des fractions lipidiques sériques, sous régime riche en acides gras polyinsaturés.

Appropriate adjustment of diet and specially reduction of the saturated fatty acids content is the cornerstone of correcting many hyperlipidemias. What we have tried in our study was first to show the evolution of the changes in the serum's fatty acids composition under a rich polyunsaturated fatty acids diet, second to find an index proving that the diet had correctly been taken. Chromatographic analysis were performed after ultracentrifugal separation of the lipoprotein fractions. Seven volunteers, non fat, and normolipemics were selected. Samples were collected every three or five days before and during the diet. The whole test lasted twenty days. What had been shown is that the linoleic acid content increased but the one of the oleic acid decreased in all lipoprotein fractions. Those changes are bestly illustrated by using the (formula: see text) ratio as a kinetic index. The triglycerid fraction modifications are faster than the phospholipid's one. Concerning the other fatty acids, the modifications are less spectacular : the palmitic acid decrease in every lipids fractions and the palmitoleic acid has a significant decrease only in the esterified cholesterol. And the stearic acid has no variation at least significatively.

[An unrecognised cause of hypercalcaemia: hypercalcaemia-hyocalcluria syndrome]. / Syndrome d'hypercalcèmie-hypocalciurie: une cause méconnue d'hypercalcémie.

A 32-year-old man with insulin-dependent diabetes secondary to chronic calcifying pancreatitis of alcoholic origin in whom hypocalciuria (22 to 88 mg/24 hours) was discovered by chance, renal function being normal. Plasma phosphate levels were between 25 and 35 mg/l and the level of parathyroid hormone was at the upper limit of normal. Cervicotomy led to the discovery of three parathyroid glands which were removed. Their weight was increased and their histological appearance normal. The fourth parathyroid was not seen. Hypercalcaemia and hypocalciuria were found during the operation and persis 3 years after, with none of the usual causes being found. This patient has a certain number of characteristics reminiscent of familial hypercalcaemia-hypocalciuria syndrome: high plasma calcium levels associated with low calciuria despite normal renal function and a plasma parathyroid level normal in most cases. The physiopathology of this syndrome remains unknown. Its course is benign, without renal complications. Partial parathyroidectomy is ineffective.

[Lingual necrosis from Horton's disease (author's transl)]. / Nécrose linguale révélatrice d'une maladie de Horton.

A patient with necrosis of the tongue was found to have Horton's disease. The other typical manifestations of this disease, though present, were overshadowed by the lingual necrosis. Recovery occurred after relatively high doses of corticoids.

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.

[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.