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The effect of treatment with simvastatin, a new HMG-CoA reductase inhibitor, has been investigated in 27 patients with primary hypercholesterolaemia. It produced a significant decrease of cholesterol and phospholipids in plasma, LDL and apolipoprotein B-containing lipoproteins. Plasma apolipoproteins B, C-III and E were also significantly lowered. The concentration of lipoprotein particles recognized by monoclonal antibodies (BL3, BL5 and BL7), associated with atherosclerotic disease, was also lowered by the treatment. Lipoproteins LpA-II:A-I were not changed, while LpA-I, which has been suggested to be the protective fraction of the apo A-I-containing lipoproteins, was slightly and inconsistently increased.

The authors report 40 cases of functionless pituitary adenomas, investigated by the same protocol and treated in the same way, between 1976 and 1982. The clinical, biological, neuroradiological features, clinical course and anatomopathological findings of this very uniform series are described. A complete immuno-histochemical study was undertaken. These findings suggest that the diagnosis of a true functionless pituitary adenoma should be based on the following criteria: --clinical and biological: absence of hormonal hypersecretion; --histological: absence or very few signs of secretory activity (little ergastoplasma, few mitochondria and Golgi apparatus); --immuno-histochemical: absence of reactivity to immunofluorescence and immuno-peroxydase methods.

The authors draw attention to the fact that the decrease in 17-hydroxycorticosteroids and 17-ketosteroids observed in patients under meprobamate treatment is probably due to chemical interference with assay methods, since normal response to metyrapone indicates that the adrenal function is unimpaired. The asthenia usually reported by these patients is due to depression and should not be blamed on deficiency of the adrenal cortex.

Compared with other hyperlipoproteinaemias, minor hypertriglyceridaemia of type IV in Fredrickson's classification (serum concentrations below 5 g/l) has been the object of few studies. From a study of 235 patients presenting with this metabolic abnormality, 64 were isolated who developed vascular lesions. Our patients' recruitment system makes it impossible for us to provide figures concerning the frequency of vascular lesions, but it enables us to present two important qualitative assessments. Firstly, a different profile seems to differentiate patients with atheromatous lesions from those with accidents of arterial thrombosis. The latter are heavier, consume significantly greater amounts of tobacco and alcohol and are significantly younger when vascular complications occur. Metabolically, they have a lower blood cholesterol/triglycerides ratio and a lower serum concentration of apolipoprotein B. In those who develop myocardial infarction compared with those who suffer from angina the cholesterol/triglyceride ratios are 1.38 and 0.91 respectively, and the serum levels of apolipoprotein B are 1.57 g/l and 1.20 g/l respectively (normal value: 1.30 g/l). Secondly, the very high frequency in our population of inaugural myocardial infarction is an original clinical finding. It provides and additional argument in support of the theory that hypertriglyceridaemia may be a contributing factor of arterial thrombosis. This study emphasizes the point that metabolically as well as clinically pure hypertriglyceridaemia is a very heterogeneous metabolic abnormality, as shown by its repercussions on the vascular system.

The use of polymorphic DNA segments as markers for the gene for the multiple endocrine neoplasia (MEN) syndrome, type 2a, allows the identification of family members at high risk for developing medullary carcinoma of the thyroid and other tumors, especially pheochromocytoma. Several families have also been identified in which medullary thyroid carcinoma is inherited, but pheochromocytoma is not seen. We have analysed 18 families, 9 with MEN 2A and 9 with medullary carcinoma of the thyroid without pheochromocytoma, with probes specific for the pericentromeric region of chromosome 10 and conclude that the mutations for the two presentations are closely situated. Genetic heterogeneity of the susceptibility locus was not seen among this sample of 18 families. The genetic mutation for medullary carcinoma was in disequilibrium with the marker alleles of the two closely linked probes, IRBPH4 and MCK2. These data suggest that different mutant alleles of the same gene or closely linked mutations account for the variation in penetrance of pheochromocytoma in families with hereditary medullary thyroid carcinoma.

The authors underline a number of features of primary hypothalamic tumours (except craniopharyngioma) based on a study of 17 cases: The vital prognosis is satisfactory; only one death was observed with an average follow-up period of 80 months (range 1 to 17 years). This raises the question of the necessity for systematic biopsy to confirm the tumour histology (although when performed in conditions of stereotaxis there are no serious risks). The site of the tumour makes surgery difficult and complete ablation impossible. As many hypothalamic tumours, including the pilocytic astrocytoma which is the commonest type, are sensitive to radiotherapy, this would seem to be the treatment of choice. Total regression is observed in the majority of cases. From the endocrine point of view, the mediocre prognosis is aggravated by high energy radiotherapy. However, with the present therapeutic methods, it is relatively easy to compensate complete or dissociated deficits. There is an endocrine syndrome suggestive of hypothalamic tumour which consists of anterior pituitary deficiency, diabetes insipidus and moderate hyperprolactinaemia (20 to 100 ng/ml). Close medical surveillance is essential, both of the local (visual field and frontal CT scanning) and endocrine effects. Hormonal deficits may occur as a late complication up to 5 years after radiotherapy. The problems inherent to hypothalamic tumours: neurogenic hypernatraemia which is luckily uncommon, with an unexplained weight gain occurring during substitute therapy for adrenal insufficiency with physiological doses of hydrocortisone. Finally, the overall prognosis is good with a minimum of complications due to radiotherapy, excepting endocrine deficiency, providing a dose of 60 grays in 6 weeks at 2 grays per session is not exceeded.

The authors present two cases of intrasellar chordoma and review these tumor's most important characters. This intrasellar localization seems to be very rare. Endocrinological disorders, when observed, leads only to insfufficiency and never to hypersecretion. Final diagnosis is established by histological studies. Their volume is important, so that the recurrence after surgery is common and the middle term prognosis is bad.

Treatment for excessive growth has alway constituted, in the endocrinological field, and particularly in the United States where such problems are frequently encountered, an incompletely solved problem, where the use of oestrogen therapy for girls has given more or less convincing results. We believe we have found a better solution which comes closer to tackling the real problem by using a minima corticotherapy, delayed until a height 1 or 2 cm short of the desired height is reached and bridging the time later required for the conjugation cartilages to fuse, under the influence of physiological puberty which is allowed to occur naturally under this method. 11 adolescents (1 boy, 10 girls) underwent this treatment at ages between 12 1/2 and 16 (average age 14 years 2 months), at a standard dose of 15 mg/day of prednisone in 3 equal doses over a period of between 7 and 25 months with periodical checking of the clinical condition, radiological bone structure and satisfactory biological tolerance. Results on growth were quite clear: growth rhythm was immediately inhibited, falling from 7,2 cm to 0,5 cm/year and there was total arrest of expected growth and a final height of about 1 or 2 cm more than the height at the onset of treatment was reached. The quality of the results obtained by this method seems to be considerably better than those obtained by other methods which have so far been suggested and this is all the more important as, provided specialised clinical and biological checks are carried out very regularly, it is not countered by unfortunate side-effects. Side-effects either do not occur at all with this method or are very minor and always regress when treatment is terminated.