Showing papers in "Annales D Endocrinologie in 1992"

[Cholecystokinin, neurotensin and corticotropin-releasing factor, three important anorexic peptides].

Abstract: This paper updates the informations on the three most important anorexigenic peptides: cholecystokinin, neurotensin and corticotropin-releasing factor. Their peripheral and/or central effects on food and water intakes as well as on dietary preferences are detailed. Their mechanisms of action and regulation are examined. This includes the interactions with classical neurotransmitters (norepinephrine, dopamine, etc...) as well as the description of the brain nuclei and neuronal networks involved. Finally, their variations in disturbed feeding behavior (hyperphagia, anorexia) in man or in animal models are reviewed.

Substernal goiters. 218 operated cases

Abstract: The authors are presenting a retrospective study on 218 retrosternal goiters operated between 1968 and 1991. 33% of the goiters were incidentally discovered on a plain X-ray of the chest. Symptoms of compression were present in 50.5% of patients and hyperthyroidism in 16.5%. Respiratory manifestations were more frequent and more severe in patients aged 70 and more. Moreover 90% of those old patients were symptomatic. Goiters migrated anteriorly in 57.7% of cases, posteriorly in 33.9%, both anteriorly and posteriorly in 5.5%. Type of migration was unknown in 2.5%. 3.7% were malignant. 27 patients with respiratory symptoms received corticosteroids to reduce the airway compression. Among 52 patients with hyperthyroidism, 36 were given antithyroid drugs. Among them, some received corticosteroid drugs in order to reduce risks of mediastinal compression. A simple cervicotomy was adequate in 92.7% of case and was completed by sternotomy in 7.3%. The operation was iterative for relapsing or forgotten thoracic goiters in 24 cases. Morbidity was slight even for sternotomized patients. Mortality was noted in 2 cases (0.9%) one of them operated on for poorly-differentiated and suffocating carcinoma of the thyroid. We advise a straightforward surgical attitude as a result of the slight morbidity and mortality, even in sternotomized patients in order to avoid severe compressive complications of the airways.

L'acromegalie familiale. a propos d'une observation. revue de la litterature

Abstract: Two brothers and a sister with acromegaly--one of them with acromegalogigantism--are described They all presented pituitary adenomas, and the diagnosis was made between the ages of 17 and 29 There was no feature of multiple endocrine neoplasia The familial investigation consisted of detailed interrogation, basal hormonal evaluation and HLA determination (haplotypes A and B) in 14 members, including the patients themselves Despite basal plasma GH levels ranging from 800 to 1000 ng/ml in 3 other family members, the normality of both GH response to induced-hyperglycemia and CT-imaging of the pituitary gland excluded the diagnosis of acromegaly in these subjects No correlation with the HLA haplotypes was observed Differential diagnosis with familial multiple endocrine neoplasia (MEN type 1) is discussed Previous reports of familial acromegaly are reviewed and analysed It appears that familial acromegaly may be considered as a specific entity for two main reasons: 1) owing to the low incidence of acromegaly in the general population, familial cases are unlikely to be fortuitous 2) most of the reported cases share some common clinical features, such as a male predominance, a high incidence of acromegalogigantism, and the presence of a pituitary macroadenoma However, the primitive disorder and the genetic transmission of the disease are still unknown

Pituitary tumor syndrome and hyperprolactinemia in peripheral hypothyroidism

Abstract: We studied 7 women with primary hypothyroidism who were suspected to harbor a prolactinoma on the basis of a pituitary gland enlargement associated with hyperprolactinemia. The diagnosis of prolactinoma was confirmed in 1 case only, which was treated surgically. In the remaining 6 patients the serum prolactin levels fell to normal on thyroxine replacement therapy and reduction in size of the pituitary gland was demonstrated by neuroradiological imaging in the 3 documented cases. This study underlines the fact that hyperprolactinemia associated with pituitary hyperplasia is secondary to hypothyroidism and that thyroid function tests should be obtained in all hyperprolactinemic patients even those with pituitary enlargement.

Hypothyroid myopathy. Physiopathological approach

Abstract: Patients with thyroid deficiency often complain of muscular weakness, exercise intolerance, cramps and excessive fatiguability. Hypothyroidism induces a metabolic myopathy, with a fall of the energetic production, and especially of the mitochondrial metabolism. This is due to a global inhibition of the main oxidative pathways (substrate incorporation, substrate oxidation) and of the respiratory chain. A diminished energetic consumption is partially related to a transition in the myosin isoforms, which express a slower ATPase, and to an impairment of the trans-sarcolemic transports. Exercise intolerance could be due to an abnormal recruitment of several metabolic pathways, such as glycolysis, related to the mitochondrial metabolism impairment, and including an abnormal accumulation of protons and monovalent phosphate ions, which are involved in the alteration of the actin-myosin interaction, and also by an abnormal Ca++ metabolism. The decreased number of NA+/K+ ATPase dependent pumps could imply an abnormal intracellular Na+ level and explain the frequent disorders of the membrane excitability.

[Catheterization of the inferior petrosal sinus. Its role in the diagnosis of Cushing's syndrome. Experience with 23 explorations].

Abstract: Selective catheterization of the inferior petrosal sinus has been performed in 23 patients: 11 Cushing's disease, 5 lung carcinoid tumors whose diagnosis has been confirmed by surgery and 7 ACTH-dependent hypercortisolism whose final diagnosis has not yet been proved. The pituitary origin of hormone secretion is accepted if the central-to-peripheral ratio (C/PR) is equal or greater than 2 on basal values and/or during CRF test. On basal values, all subjects but one with confirmed pituitary lesions exhibited a C/PR > or = 2; the carcinoid tumors never exceeded the value of 1.3. After CRF injection, all the pituitary tumors showed an increase in C/PR; one patient with a lung carcinoid tumor (CRF-negative) showed a pituitary ACTH secretion (C/PR = 3.7). The localization of pituitary adenoma by the lateralization of hormone secretion is disappointing, except, perhaps, for very small extremely lateral adenomas. No accident occurred in our series, nor in the literature. This investigation seems the most reliable approach to prove the pituitary origin of hormonal hypersecretion. We recommend it to be performed in ACTH-dependent Cushing's syndrome when the secretory pattern is not typical and/or pituitary imaging is normal.

The influence of oxytocin, vasopressin and their analogues on progesterone and testosterone production by porcine granulosa cells in vitro

Abstract: Effects of nonapeptide hormones and some of their chemical analogues on progesterone and testosterone production by culture of porcine granulosa cells have been investigated. Oxytocin (0.01-10 IU/ml), arginine-8-vasopressin (0.01-10 micrograms/ml), arginine-8-vasotocin (0.01-10 micrograms/ml) and, in a lesser degree, 2-0-methyl-tyrosine (deamino-1-karba)-oxytocin (0.01-10 micrograms/ml, but no 1-deamino-8-vasopressin (0.01-10 micrograms/ml) stimulated a progesterone surge. Testosterone production was significantly stimulated by oxytocin and inhibited by vasopressin or vasotocin additions. 2-0-methyl-tyrosine (deamino-1-karba)-oxytocin or 1-deamino-8-vasopressin had little or no effect on testosterone secretion. The present results suggest the existence of a direct influence of nonapeptide hormones on porcine ovarian progestagen and androgen production.

Marijuana and reproduction. Effects on puberty and gestation in female rats. Experimental results

Abstract: The main psychoactive component of marihuana, delta-9-tetrahydrocannabiol (THC), was investigated at low doses (1 microgram/kg/day) on the onset of puberty, on the reproductive functions in female rats up to the seventy fifth to eightieth day of life as well as during the pregnancy. The administration of THC caused a delay of the onset of puberty, and the number of ova on the day of first estrus was significantly lower in treated animals. After puberty, alterations occurred in the neuroendocrine functions of animals received THC: estrous cycles were irregular, serum LH level was decreased. When THC was injected during the third week of pregnancy it caused a significant prolongation of the gestation period. There was 30% stillbirths (v.s. 3% in physiological saline treated rats). No teratological effects were observed. Serum LH, progesterone and prostaglandin contents were decreased. The results indicate that marihuana causes alterations in reproductive functions. The importance of fight against drug abuse is emphasized.

Increase of cancers of the thyroid gland in children in Byelarus

Abstract: Last September, "Nature" reported a great increase in the frequency of thyroid cancer in children in Belarus. Some additional data are now available and strengthen the previous findings. The overall incidence of thyroid cancer in children rose from an average of four cases per year till 1989 to more than 50 in 1991 and 1992. Nearly all of the 146 cancers recorded since 1986 are papillary carcinomas. Moreover these cancers appear relatively aggressive. The occurrence of such an increase within a few years of exposure--and only in children--is quite unexpected. Therefore, it seems very important to monitor trends in future and more information is needed, as different questions remain unanswered: dosimetry, diagnosis methods, other thyroid diseases... In conclusion, the authors stress on the very importance of iodine prophylaxis in case of an accidental contamination by radioisotopes of iodine. It seems possible that cancer excess would have been less important, if Belarus children had been given stable iodine.

Reciprocal control of oxytocin and cAMP production by porcine granulosa cells in vitro

Abstract: Oxytocin and cAMP accumulation in the medium after 4 days incubation of porcine granulosa cells was measured and confirmed the production of these substances by porcine ovaries. Both cAMP analogue dbcAMP (10, 100, 1,000 or 10,000 microM/ml) and 3-isobutyl-1-methyl-xanthine (intracellular cAMP metabolization inhibitor, 0.1, 1, 10 or 100 microM/ml) additions increased granulosa cells oxytocin production in a dose-dependent manner. Low doses of oxytocin or posterior pituitary extract (10 or 100 mIU/ml) had no significant effect on granulosa cAMP output, but higher dose (1,000 mIU/ml) inhibited it. Thus, porcine ovarian oxytocin production can be stimulated via cAMP-dependent intracellular mechanisms. On the other hand, excess of oxytocin can inhibit ovarian cAMP production through a ultra-short loop negative feedback regulation.

Place de la scintigraphie corticosurrénalienne dans l'exploration des incidentalomes.

Abstract: To determine the utility of adrenocortical scintigraphy with I131-6 beta-iodomethyl-19-nor-cholesterol (NP59) in incidentally discovered adrenal masses, we studied 12 patients with a unilateral adrenal mass and without other primary tumors or signs of pheochromocytoma or hyperfunctioning adenoma. Ten patients had an adenoma (size: 12 to 35 mm), the diagnosis was made by surgery or by no change in size on repeated CT scans. The NP59 scintigraphy showed an increased uptake on the side of the tumor in 8 cases with a decreased uptake of contra-lateral gland in 7 cases. Hormonal investigations of glucocorticoid function suggested supranormal or fluctuant cortisol secretion in 5 cases as assessed by moderately elevated urinary free cortisol or by incomplete dexamethasone suppression test. These abnormalities disappeared after surgery. Two patients had normal bilateral uptake of NP59, the sizes of the tumors were 12 and 20 mm. Two patients had an extra-adrenal tumor. The NP59 scintigraphy showed a moderately decreased uptake on the side of the hematoma of one patient and a compression of the normal adrenal by ganglioneuroma of the other patient. Our results and those of other authors suggest that positive NP59 scintigraphy could confirm the cortical nature of an incidentally discovered adrenal mass, probably an adenoma that must be followed up morphologically and functionally. No uptake by a tumor greater than 2 cm suggests a primary malignancy or extra-adrenal origin which must be diagnosed by invasive methods.

Insulin resistance and polycystic ovary syndrome

Abstract: The causes and consequences of the insulin resistance that accompanies the polycystic ovary Syndrome (PCOS) are still controversial. The hyperandrogenism does not appear any more as a causal factor but on the contrary is likely to be a consequence, via the ovarian effects of hyperinsulinism. These are still not clearly understood in their molecular aspects, but their reality is undoubtful in view of some clinical studies which showed an improvement following by the reduction of hyperinsulinism. Therefore, the aetiopathogenic role of the insulin resistance in PCOS becomes more and more obvious but it cannot be split from the likely implication of the intra-ovarian growth factors, and more especially the IGF 1 which could act synergistically with it. Moreover, the presence of LH seems to be still a pre-requisite to the ovarian action of insulin. The therapeutical incidences of these hypothesis are obvious and should improve in the next future the medical management of PCOS.

Influence of dose and modalities of administration of BIM 23014 on growth hormone secretion in patients with acromegaly

Abstract: Somatostatin analogs, with prolonged half-lives have been proposed for the treatment of acromegalics. The aim of the study was to evaluate the short term efficacy of different doses and modalities of administration of the new somatostatin analog, BIM 23014 (BIM), on GH secretion in acromegalics. Ten acromegalics, with evolutive disease, who previously had had transsphenoidal surgery (and pituitary radiotherapy in 8) were evaluated in a three step study. The first part included four patients who received in a random order either vehicle or 500, 1000 and 1500 micrograms BIM for a day as a continuous s.c. infusion using programmable pumps at one-week interval for 24 hours to measure plasma GH levels. The second part included six patients who received in a random order either vehicle or 1500 micrograms/24h BIM as 500 micrograms x 3 s.c. injections, 750 micrograms x 2 s.c. injections and a continuous s.c. infusion using programmable pumps at one-week interval. During each period of the study blood was sampled at 4 hour intervals for 24 hours in order to measure plasma GH and BIM levels by radioimmunoassays. The third part of the study included the same 6 patients as the second part, who received 30 mg IM of a long acting formulation of BIM. Blood was sampled before and thereafter on days 1, 3, 6, 9, 12, 15, 18 and 21 following the injection for measurement of plasma GH and BIM levels. In first group 500 micrograms BIM slightly decreased plasma GH levels.(ABSTRACT TRUNCATED AT 250 WORDS)

Blocs enzymatiques surrénaliens à révélation tardive par déficit de la 11-hydroxylase. A propos de 29 observations.

Abstract: 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.

Influence of the maternal pineal gland on the testicular function in rats

Abstract: Female rats were exposed to a short (6L:18D) photoperiod from 21 days of age. On day 2 of gestation, animals were pinealectomized or sham-operated. Lighting regimens were not changed during the course of the study. Male offspring of the 2 groups of rats were sacrificed on days 21 after birth. Pinealectomy of the mother induced the following modifications: a decrease in prostate and seminal vesicle weights, a decrease in testicular testosterone and androstenedione content and in plasma dihydrotestosterone levels. We conclude that when rats are maintained on a 6L:18D cycle the maternal pineal gland stimulates the testicular function in 21-day-old offspring.

Acromegaly and bronchial carcinoid tumor. Apropos of a case

Abstract: Acromegaly due to ectopic secretion of growth hormone-releasing hormone (GHRH) is rare. A 29-year-old woman with acromegaly secondary to ectopic GHRH secretion by a bronchial carcinoid tumor is presented. Normalization of GHRH levels, reversal of pituitary hyperplasia and regression of acromegaly followed resection of the bronchial tumor. The authors stressed the interest of GHRH dosage in patients with acromegaly and pituitary hyperplasia.

[Biological and developmental aspects of macronodular adrenal hyperplasia in Cushing's disease].

Abstract: The pathophysiology of macronodular adrenocortical hyperplasia (MNH) associated with Cushing's diseases remains debatable. Some authors have claimed that MNH represents an autonomous condition that has to be treated by surgical adrenalectomy while others advocate the use of pituitary surgery. We have compared the biochemical features, before and after pituitary surgery, between 7 cases of MHN and 22 cases of diffuse hyperplasia (DH) associated with Cushing's disease. In addition, the diameter of the adrenal nodules after surgery was evaluated in 6 cases. The mean ACTH level in MNH and DH subjects was similar. Dexamethasone suppressibility and the stimulatory effect of metyrapone on ACTH secretion were less in MNH than in DH subjects, suggesting a greater degree of adrenal autonomy in the former. The cortisol responses to metyrapone and CRF tests suggested an increased responsiveness of the adrenals to endogenous ACTH in the MNH group. Only one MNH patient had biochemical features suggesting a primary adrenal disease. The use of a sensitive ACTH assay and the results of inferior petrosal sinus sampling identified the ACTH-dependency of the disease. A short to medium term remission of the disease was obtained in all cases of MNH and in 87% of cases of DH after surgery. The diameter of the adrenal nodules significantly decreased in only 43% of cases. Our results suggest that the adrenal autonomy in MNH is incomplete and that, despite the persistence of the adrenocortical nodules, the disease can be cured with pituitary microsurgery in most cases.

[Contribution of intra-operative measurement of intact parathormone in surgery for primary hyperparathyroidism].

Abstract: Using a sensitive two-site immunoradiometric assay which detects intact parathormone (iPTH), we studied the decrease in peripheric and jugular plasmatic iPTH during surgical removal of abnormal parathyroid (s). In the next future, results of intact parathormone (iPTH) assay will be given in 45 minutes. In a prospective study of 33 patients operated on for hyperparathyroidism or for cold thyroid nodule, the serum levels of intact PTH was measured intraoperatively in peripheric and in jugular blood. The preoperative mean serum iPTH concentration was 119.23 +/- 172.48 pg/ml and fell to 34.5 +/- 32.21 pg/ml after surgery in 14 cases of primary hyperparathyroidism (p < 0.001). Thirteen out of 14 patients had serum iPTH values less than 65 pg/ml within 15 minutes after parathyroidectomy. The preoperative mean serum iPTH concentration in the 5 secondary hyperparathyroidism was 781.2 +/- 403.19 pg/ml. This value fell to 124 +/- 66.91 pg/ml after parathyroidectomy (p < 0.04). No significant decrease was observed in the mean serum concentration of the 14 patients operated on for cold thyroid nodule. Patients suffering from single parathyroid adenoma presented a significant gradient in jugular plasmatic PTH concentration between the adenoma side and the contralateral one. This gradient decreased during effective parathyroid adenomectomy (309.7 +/- 313.3 pg/ml to 3.7 +/- 35.1 pg/ml). Intraoperative serum iPTH concentration will provide a valuable tool to appreciate the effectiveness of surgical removal of parathyroid glands and to detect the location of parathyroid adenoma when the surgical research is negative.

Contribution of chemiluminescence for determination of low value TSH in patients with dysthyroidism during negative TRH test

Abstract: TSH values performed by immunoradiometric method were compared with chemiluminescent method in 28 hyperthyroid patients with negative TRH-test. This last method gives a better sensitivity (0.020 mUI/L vs. 0.050 mUI/L) and reproducibility (5% V.C. vs. 40%). The measure of undetectable TSH by immunoradiometric assay becomes possible with chemiluminescence. Due to this increase of sensitivity, the TRH-test needs to be reconsidered and allows to discriminate various degrees of TSH suppression.

Epidemiology of goiter in Auvergne

Abstract: An epidemiological study about goiter, related to the French national survey concerning school children aged 10-16, was carried out in region Auvergne. The prevalence of goiter was 12.52%, including 2.8% patients with WHO stages I and II, thus being slightly below national average (16.7%). Iodine urinary excretion was 62.9 +/- 34.3 micrograms per gram creatinine (French mean value 85.0), although sometimes major variation was noted in the same area. Later, two studies were performed in adults, in two places of Haute-Loire and Puy-de-Dome, respectively Arsac-en-Velay and Besse-en-Chandesse. Both places were selected because of high frequency of goiter in children. Prevalence in adults was 16.1% in Arsac-en-Velay including 12.5% stages I and II, and 8.9% in Besse-en-Chandesse including 5.9% stages I and II. Mean iodine urinary excretion was respectively 59.78 micrograms/gram creatinine and 43.45 micrograms/liter. Thus, these surveys confirm a significant prevalence of goiter in Region Auvergne, with endemic places, and a generally low iodine urinary excretion.

Prolactin-like substance secretion by granulosa cells isolated from bovine ovaries.

Abstract: By the 5-day culture of bovine granulosa cells both in serum-free and in serum-supplemented medium the time-dependent accumulation of PRL immunoreactivity was observed FSH additions (10-10,000 ng/ml medium) led to a dramatic rise of immunoreactive PRL in a dose-dependent manner LH stimulated the increase of PRL-like substance only in a great dose (10 IU/ml) Lower LH doses (001-1 IU/ml) had no significant influence on this process Low doses of oxytocin (1 or 10 mIU/ml) blocked, and higher ones (100 or 1,000 mIU/ml) stimulated the granulosa PRL-like substance accumulation Arginine-8-vasopressin (1-1,000 ng/ml), arginine-8-vasotocin (10-10,000 ng/ml), or LH-RH (10-10,000 ng/ml) failed to influence the PRL immunoreactivity accumulation in the culture medium Present data may suggest the production of PRL-like substance by bovine ovarian cells, as well as the involvement of gonadotropins and oxytocin in the regulation of this process

[Partial 3-beta-hydroxysteroid dehydrogenase deficiencies].

Abstract: An overview of the partial 3 beta-hydroxysteroid dehydrogenase deficiency is presented. The classical, congenital or early postnatal form is characterized by a salt-losing syndrome and/or ambiguous genitalia. The late-onset forms, only recognized for the last ten years and diagnosed with an increasing frequency, are to be systematically suspected in the presence of clinical hyperandrogenism with or without oligomenorrhea. This deficit, involved in both adrenal and gonadal tissues, seems to be transmitted by an autosomal recessive gene. An ovarian 3 beta-hydroxysteroid dehydrogenase deficit can be a primary cause of some cases of polycystic ovary syndrome and the relations with this affection are disputed. The increased ratios of delta 5 steroids/delta 4 steroids ensure the diagnostic conviction while the elevated ratio of 17-hydroxy-pregnenolone/17-hydroxyprogesterone and the normal ratio of 11-desoxycortisol/cortisol allow to eliminate the possibility of a 21-hydroxylase or 11 beta-hydroxylase deficiency, respectively. The treatment is based above all on the glucocorticoid utilization, which can lead to the return of menses and the ovulatory function, but the cutaneous symptoms of hyperandrogenism will be better controlled by cyproterone acetate out of situations of stress.

[Genetic susceptibility of insulin-dependent diabetes].

Abstract: Type I Insulin-Dependent Diabetes Mellitus (IDDM) is an autoimmune disease characterised by the destruction of pancreatic Beta cells. There is an evidence for a contribution of genetic factors to the development of the disease and it is widely documented that HLA antigens contribute to the disease susceptibility. HLA-DR3 and HLA-DR4 associations have been firstly described in Caucasian. Recent studies at the gene level have elucidated this HLA association more precisely, pointing out the prominent role of HLA-DQ locus genes. The hypothesis has been proposed that some critical amino-acid at position 57 of DQ Beta chain and at position 52 of DQ Alpha chain both contribute to disease susceptibility. According to the functional role of HLA class II molecules, these particular residues may affect the antigen binding and T cell recognition and therefore contribute to the triggering of the pathological auto-immune response.

[MRI exploration of the hypothalamo-hypophyseal axis].

Abstract: MR Imaging has become the procedure of choice for the exploration of the pituitary region. MR contrast resolution is far better than the finest CT examination, especially concerning the microadenomas and the neurohypophysis. The present paper concerns, at first the major principles of magnetic resonance imaging, its application for the study of the pituitary region including the use of contrast agent (Gadolinium). Lastly, MR anatomy and physiological variations are presented.