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[Functional morphology of adenohypophysis, thymus, and adrenal cortex in sudden infant death syndrome].

Adrenal glands, hypophysis and thymus of infants (mean age-4 months), who died of SIDS (30 cases), AVRI (10 cases) and violence (4 cases) were studied by morphometrical methods. The following changes in SIDS (in comparison with AVRI and violent death) were observed: a decrease of hypothesis weight with decreased quantity and size of basophil cells; reduced adrenal weight with thinning of the cortex particularly in the fascicular zone; diminished volumes of the nuclei in the adrenocortical cells of the fascicular zone; thymomegaly with hyperplasia of the thymus cortex; increased number of correlation links between morphometric indices of hypophysis and adrenal cortex. These changes may be indicative of a subclinical hypophyseal-adrenal insufficiency in SIDS. Adrenal cortex hypofunction was indirectly confirmed by the presence of thymomegaly. Long-lasting glucocorticoid deficiency in secondary hypocorticoidism may cause sudden infant death following minor environmental causes.

Adrenal Cortex↗

[Adrenocortical function in myasthenia pseudoparalytica].

In a group of 31 women with myasthenia the urinary excretion of 17-ketosteroids and their fractions (11-deoxy-17-ketosteroids, dehydroisoandrosterone) and 17-hydroxycorticosterone were determined. A statistically significant decrease was observed in the excretion of these substances. Besides that, the plasma cortisol level was determined in these patients and the results obtained in the whole group were at the lower normal range of the laboratory and in 40% of cases they were significantly lower (p less than 0.01). The obtained results may suggest that adrenocortical hypofunction exists in many cases of myasthenia. The pertinent literature is reviewed.

17-Hydroxycorticosteroids↗

Polyglandular autoimmune (PGA) syndromes: report of three cases and review of the literature.

Polyglandular autoimmune (PGA) syndrome is caused by autoimmune process in multiple endocrine glands. This usually results in endocrine gland hypofunction, except for the thyroid gland, in which both hyper or hypofunction may occur. The syndrome can be classified into two types, type I and type II, each with distinct clinical characteristics. We report three cases of PGA syndromes. The first patient had type I PGA syndrome, characterized by hypoparathyrodism, primary adrenal insufficiency and primary ovarian failure. She also had chronic mucocutaneous candidiasis, which is the distinct feature in this syndrome. The second patient had type II PGA syndrome, with primary adrenal insufficiency, Hashimoto's thyroiditis and primary ovarian failure. She also had widespread vitilgo. The last patient also had type II PGA syndrome. She had insulin dependent diabetes mellitus, Graves' disease and alopecia areata.

Adult↗

[Diabetic feto- and embryopathies].

The present article gives an analysis of available literature and the authors' own findings. Perinatal mortality of progeny of mothers suffering from diabetes mellitus may be caused both by embryopathy and antenatal fetopathy. The authors did not observe diabetic gameto- and blastopathies and no indications to their existance was found in literature, although some investigators admit the possibility of genetic implication in the pathology of progeny of parents with diabetes mellitus. In the progeny of mothers suffering from diabetes mellitus among congenital malformations which allow further intrauterine development and even extrauterine life, according to literature data most often there were observed malformations of the skeleton (37%), cardiovascular system malformations (24%) and those of the nervous system (14%). In all observations of the authors there were found congenital malformations of the bone system not infrequently in combination with other congenital defects. The main features of diabetic fetopathy appear in the last third of pregnancy, and therefore it may be considered as late antenatal fetopathy. The term "diabetic children" applied by some authors to children with diabetic fetopathy should be avoided since it may be erroneously understood as diabetes mellitus. In the majority of cases quite different mechanisms underlie these conditions: hypofunction of the islet apparatus of the pancreas in diabetes mellitus, and its hypofunction in diabetic fetopathy. Diagnosis of diabetic feto- and embryopathies is always based on both clinical and morphological data.

Abnormalities, Multiple↗

[Characteristics of immunologic and neuro-hormonal status in patients with neuroses].

More than 3500 neurotic patients have been examined with emphasis on their immune status and functioning of the sympathoadrenal system (SAS) (determination of adrenaline, noradrenaline, dopamine and L-DOPA excretion in terms of their daily biorhythm). The comparison of the immune and neurohormonal data of neurotic patients indicated an obvious relation between the immune status and the type of SAS functioning. The high-risk group comprises patients with the adrenaline type of SAS functioning and pronounced adrenaline and noradrenaline hypersecretion, SAS hypofunction, and imbalance between the hormone-mediated and immunologic characteristics.

Adrenal Glands↗

Plasma cortisol is often decreased in patients treated in an intensive care unit.

OBJECTIVE: To investigate the prevalence of adrenal hypofunction, as assessed by plasma cortisol (p-cortisol) and its relationship to clinical events. DESIGN: Prospective, consecutive. SETTING: General intensive care unit in a university hospital. PATIENTS: Fifty-five patients (34 men and 21 women) were studied (surgery 40 patients, hemodialysis 5, ventilator treatment 45, sepsis 21). METHODS: Morning basal levels of p-cortisol were determined. Previous reports define adrenal insufficiency to be present if p-cortisol under stressful conditions is lower than either 400 or 500 nmol/l. The tetracosactoid test (250 microg Synacthen) was performed in 16 patients and urinary 24-h excretion of cortisol in 24 (none on corticosteroid treatment). RESULTS: Median p-cortisol was 550 nmol/l (range 20-1764). In 36% of patients p-cortisol was lower than 400 nmol/l and in 47% lower than 500 nmol/l. There was a significantly increased probability (P < 0.05) of p-cortisol being below 400 nmol/l in patients admitted due to trauma or cerebral disorder and in patients on ventilator therapy or on mannitol. Thirty minutes after tetracosactoid administration p-cortisol response was lower than 200 nmol/l in 56% of the patients. CONCLUSIONS: Several patients had low p-cortisol and attenuated responses to tetracosactoid, indicative of adrenal insufficiency. There seem to be certain risk factors for adrenal hypofunction which may justify more frequent use of physiological doses of corticosteroid in selected patients.

Adolescent↗

[Pituitary apoplexy: analysis of endocrine function in 17 cases].

BACKGROUND: From a neurological standpoint, pituitary apoplexy (PA) is a well defined syndrome. There are few systematic studies addressing pituitary hormone secretion after a PA episode. The aim of the present study was to assess the frequency and degree of endocrine dysfunction due to PA. METHODS: In 17 consecutive patients, the secretion of growth hormone (GH), the pituitary-adrenal axis status, thyrotropin (TSH), prolactin and gonadotropins (LH, FSH) were evaluated after the administration of insulin, thyrotropin releasing hormone (TRH) and gonadotropin-releasing hormone (LHRH) after an episode of PA. 20-90 days after surgery the measurements were repeated. Antidiuretic hormone (ADH) was measured by plasma/urine osmolality after water deprivation and, in some cases, by administration of hypertonic saline. RESULTS: The most commonly found deficiency was that of GH (84%), which in two cases resulted in cure of acromegaly, followed by that of LH (78%). Pituitary-adrenal dysfunction was improved in two patients after surgery. In all cases except one there was a reduced secretion of at least two hormones. If serum prolactin was reduced, the rest of pituitary function was usually impaired. In one case, permanent diabetes insipidus developed after PA. The prevalence of PA in pituitary adenomas was 9%. CONCLUSIONS: Pituitary hormone secretion after a PA episode is almost invariably impaired. This impairment may be reversed after surgery. Hypoprolactinemia is an indicator of pituitary hypofunction.

Adenoma↗

Adrenocortical hormone levels during cardiopulmonary bypass with and without pulsatile flow.

To determine the effect of hypothermic pulsatile and nonpulsatile cardiopulmonary bypass (CPB) with hemodilution on adrenocortical function we measured plasma levels of adrenocorticotropic hormone (ACTH), cortisol, aldosterone, and renin in two groups of patients. Group I, comprising 11 patients had routine CPB (nonpulsatile), and Group II, comprising 12 patients, had pulsatile flow during CPB (pulsatile). Both groups demonstrated comparable increases in cortisol, ACTH, and aldosterone with operation. Levels for all three hormones appeared to decline during CPB and then rose again in the post-CPB period. There were no significant differences between groups. Plasma renin activity gradually declined in a comparable manner in both groups. In the post-CPB period, renin activity was slightly higher in the nonpulsatile group (1.7 +/- 0.5 versus 0.8 +/- 0.2 ng/ml/hr, p less than 0.05). Correction for the effect of hemodilution demonstrated no decrease in cortisol and a slight increase in ACTH in both groups during CPB. Significant increases occurred in both groups during CPB in urinary Na+ excretion rate and urinary Na+/K+ ratio, more so for the nonpulsatile group. There was no correlation between urinary Na+/K+ ratios and either plasma cortisol or aldosterone levels. Thus routine CPB demonstrates no evidence of adrenocortical hypofunction and the addition of pulsatile flow produces little improvement.

Adrenal Cortex Hormones↗

[Disorders in the hypophysis-adrenal and hypophysis-thyroid systems of patients with acute pneumonia and the ways for their therapeutic correction].

Using RIA, 188 patients with acute pneumonia were examined over time for the blood ACTH, cortisol, TTH, T3 and T4 content as well as for T4/T3, TTH/T3 and TTH/T4 correlations. Under discussion are causes, approaches to the prevention and correction of the hormonal disorders revealed in the pituitary-adrenal system in patients with an unfavourable course of pneumonia. The majority of the patients manifested thyroid hypofunction and TTH hypersecretion, which remained unchanged even during the clinical recovery, since the first days of the disease. It is advisable that microdoses of thyroidine may be included into the multimodality treatment of patients afflicted with acute pneumonia and iodine-containing expectorants excluded from it.

Acute Disease↗

[A case of lymphocytic adenohypophysitis associated with sarcoidosis of the lung and eye].

Lymphocytic adenohypophysitis is considered to be an inflammatory disease of the adenohypophysis that is commonly present with visual disturbance and hypopituitarism. Its etiology remains unclear but it is often related to an autoimmune disorder involving other organs, such as the thyroid, parathyroid, or adrenal glands. We encountered a rare case of lymphocytic adenohypophysitis associated with sarcoidosis of the lung and eye during the follow-up period. A 23-year-old woman was hospitalized in July 1986, with a one-month history of headache and visual disturbances which began three days after her second normal delivery. On admission, she showed slight visual impairment and had a left temporal superior quadrantanopia. Endocrinological evaluation revealed thyroid and adrenal hypofunction, and low response of human growth hormone to the loading test. A skull X-ray showed normal shaped sella with some erosion of the dorsum. CT scan showed a rounded contrast-enhanced intrasellar mass extending into the suprasellar cistern. MRI (SR: 500/30) showed a homogeneous low intensity mass which contained a small high intensity area on the relative T2-weighted image (2000/50). A biopsy was performed via right frontotemporal craniotomy. The consistency of the resected tissue was firmer than that of pituitary adenoma. Histologically, the tissue showed diffuse lymphocytic infiltration with some normal adenohypophysis. Her postoperative course was uneventful and the visual impairment improved two months later after the operation. Six months after the operation, she was readmitted with complaints of general fatigue and breathlessness. Chest X-ray showed diffuse infiltration throughout both lung fields, but there was no bilateral hilar lymphadenopathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Hypofunction of peripheral target-gland after radiotherapy of pituitary adenoma--an analysis of 140 cases].

One hundred and forty cases of pituitary adenoma, treated by surgery plus radiotherapy or radiotherapy alone from 1978 to 1985, were analysed as to the incidence of hypothalamo-pituitary and peripheral target-gland hypofunction after radiotherapy. The incidence of hypothyroidism, hypogonadism and hypoadrenalism was 51.7%, 61.4% and 17.8%, respectively. Thyroid and gonad were vulnerable following hypothalamo-pituitary hypofunction, and the incidence was 19.2% and 23.5% individually, and 27.1% simultaneously. The incidence of hypoadrenalism was 7.1%. The total incidence of target-gland hypofunction was 10.7%. Hypofunction of the posterior lobe of the pituitary often occurred after operation. For patients treated with radiotherapy alone, the incidence of hypothalamo-pituitary and target-gland hypofunction was high because of higher irradiation dose. Hormonotherapy was successful in the treatment of hypopituitarism and target-gland hypofunction. For radiotherapy of pituitary adenoma, DT 40-45 Gy and treatment field 4 x 4 cm are recommended.

Adenoma↗

[Functional indices of the hypophysis, gonads and adrenal cortex during the treatment of patients with gonorrheal inflammation of the scrotal organs using proteolytic enzymes].

Chymotrypsin, a proteolytic enzyme included in combined therapy of 27 patients with gonorrhea complicated by the scrotal organs inflammation, has normalized the functions of the hypophyseo-gonadal and hypophyseo-adrenal systems, i.e. hypophyseal luteotropic function, adrenocortical gluco- and mineralocorticoid functions, and testicular androgenopoiesis. Hyperprolactin- and hyperprogesteronemia persisted after therapy, hyperestrogenism and follitropic hypofunction of the hypophysis have developed, that may become the reason of stable pathospermia and disordered fertility after therapy of complicated gonorrhea.

Adrenal Cortex↗

Quantitative study of pulmonary endocrine cells in anencephaly.

Quantitative studies of pulmonary endocrine cells in 8 male autopsy cases of anencephaly were performed. The densities of pulmonary endocrine cells were expressed as the number of the total argyrophil cells/100 bronchiolar cell nuclei and as the number of the total argyrophil cells/mm bronchiole. The average values in the 8 anencephalic cases were 1.26 cells/100 nuclei and 3.56 cells/mm, and did not differ from the 1.28 cells/100 nuclei and 3.24 cells/mm in the control cases without malformation of the brain and without severe hyaline membrane disease of the lung. Furthermore, there was no significant difference in the densities of neuroepithelial bodies between the 2 groups. Immunostaining for calcitonin, bombesin, adrenocorticotropic hormone, chromogranin, and neuron-specific enolase also revealed no qualitative differences between them. It is concluded that defect and hypofunction of the hypothalamus-pituitary-adrenal axes associated with anencephaly do not have significant effects on the proliferation and differentiation of pulmonary endocrine cells in the fetus.

Anencephaly↗

Diseases of the adrenal cortex.

The adrenal cortex is functionally a three-dimensional gland that secretes glucocorticoids, mineralocorticoids, and sex steroids. Of these three classes of steroids only the gluco- and mineralocorticoid hormones are necessary to sustain life. The availability of sensitive and specific radioimmunoassays has permitted accurate measurement of practically every steroid hormone secreted by the adrenal cortex. As in other endocrinopathies, suppression studies are employed when hyperfunction is suspected, while provocative tests are used to detect hypofunction. These dynamic studies enable the clinician to evaluate the functional status of the adrenal cortex. The anatomic configuration of the adrenal cortices is delineated by high-resolution computed tomography (and magnetic resonance imaging), obviating the need for invasive procedures such as venography or arteriography. The disorders of the adrenal cortex can be viewed from the dual perspectives of hyperfunction and hypofunction. Clinical expressions of hyperfunctional adrenocortical syndromes include Cushing's syndrome, primary hyperaldosteronism, and the adrenogenital syndrome. The expressions of hypofunctional syndromes include Addison's disease and selective hypoaldosteronism. The diagnosis and treatment of these disorders are outlined in this issue.

Addison Disease↗

Albright's syndrome with acromegaly and Hashimoto's thyroiditis: report of a case.

The case of a 35-year-old woman with Albright's syndrome, acromegaly and Hashimoto's thyroiditis is presented. She had noted deformity of the left mandible and chest from childhood. She developed persistent galactorrhea and amenorrhea after the delivery of her second child. X ray of the skull, and a head CT, revealed a pituitary tumor and fibrous dysplasia of the left mandible, sphenoid, zygomatic bone and pteryoid plate. Serum GH and PRL levels were markedly elevated. She received recontouring surgery of the left mandible, and a pathological examination confirmed the diagnosis of fibrous dysplasia. Chest X ray also showed fibrous dysplastic change of the left 4th, 5th, 6th and 7th ribs and left clavicle. Because of poor response to bromocriptine, she received a craniotomy to remove the pituitary macroadenoma. Pathological examination of the tumor revealed an acidophilic tumor. Postoperative radiotherapy was given for residual active tumor. She developed adrenal crisis two months after radiotherapy when she discontinued replacement therapy. The diagnosis of Hashimoto's thyroiditis was arrived at by palpation of the goiter, elevated thyroid antibodies, ultrasound pictures of the thyroid, fine needle aspiration cytology and hypothyroidism. To our knowledge, this is the first report of Albright's syndrome with Hashimoto's thyroiditis. The hypothesis of autoimmune disease is proposed to explain the hypofunction of the endocrine glands associated with Albright's syndrome.

Acromegaly↗