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PubMedDate not supplied

The renin-angiotensin-aldosterone system in primary and secondary hypertension.

Important advances have been made in understanding the role of the renin-angiotensin-aldosterone system in the pathogenesis and diagnosis of hypertensive disorders. Measurement of plasma renin activity (PRA) and aldosterone is very important in the assessment of secondary hypertension. Hypertensions with increased PRA include renovascular hypertension, some cases of unilateral and bilateral renal parenchymal disease, malignant hypertension, hypertension associated with oral contraceptive agents, and renin-secreting tumors. Hypertension with decreased PRA is observed in four recognized types of primary aldosteronism: adenoma, bilateral hyperplasia, indeterminate aldosteronism, and glucocorticoid-responsive aldosteronism. Other conditions with hypertension and depressed PRA include ACTH and DOC secreting tumors, primary hyperpituitarism, syndromes of 17-hydroxylase and 11-beta-hydroxylase deficiency. Liddle's syndrome, licorice abuse, exogenous administration of mineralocorticoids, and preeclampsia.

Acute Kidney Injury↗
PubMed1997

[Osteoporosis during development--selected problems].

In children and the youth it is secondary osteoporosis (OP) rather than idiopathic one which occurs more often; its multidirectional pathogenesis is usually ascertainable. Secondary OP, mostly generalized, is diagnosed in the course of such hormonal disturbances as: primary hyperparathyroidism, hyperthyroidism, hyperadrenalocorticalism, hyperpituitarism (with excess of growth hormone) and in hypogonadism. Another group of diseases implicating OP are connective tissue pathologies: congenital (osteogenesis imperfecta, collagenopathies) and acquired (Juvenile chronic arthritis). A serious problem for a pediatrician is the iatrogenic OP resulting from a long-term use of some medicines (glucocorticosteroids, hydantoin derivatives, barbiturates), or long-lasting immobilization for surgical and orthopaedic reasons, or from chronic general diseases. Osteoporosis accompanying pathological states of the skeletal and nervous systems (with paralyses and pareses) is particularly intensive and difficult for treatment. Osteoporosis in developmental age may cause disturbances in natural development of the skeleton, which leads to deformities in the skeletal system and to the formation of faulty postures. Lower body height is a frequent complication resulting from OP in children and the youth.

Adolescent↗
PubMed1976

Transsphenoidal neurosurgery of intracranial neoplasm.

Because of the benign, rapid, and nontraumatizing nature of the extracranial transsphenoidal approach to the base of the skull, this procedure is indicated as the method of choice in nearly all cases of surgery for pituitary fossa and parasellar region access. Some of the largest pituitary chromophobe adenomas with voluminous suprasellar expansion have been successfully excised from below, as well as some calcified tumors, craniopharyngiomas, Rathke's pouch cysts, chordomas, chondromas, and meningiomas. At the other extreme, the smallest intrapituitary microadenomas (as little as 3 mm in diameter) were selectively removed under optic magnification with the surgical microscope. This method is now the most appropriate for the treatment of oversecreting pituitary disorders, even with normal-sized sella turcica (acromegaly - Cushing -galactorrhea). Early detection of intrapituitary microadenoma allows achievement of its selective excision with preservation of the normal gland. Thus, the ideal goal can be achieved by immediate biological cure of hyperpituitarism with preservation of other pituitary functions, resulting in normal physiological eupituitarism.

Acromegaly↗
PubMed1985

Corticotropin- and growth hormone-releasing factor (CRF and GRF) in the diagnosis of hypothalamo-pituitary diseases.

The recently discovered releasing factors for ACTH and growth hormone, CRF and GRF, have stimulated our understanding of the hypothalamo-pituitary physiology and pathophysiology. Whereas CRF, a 41 amino-acid residue peptide, seems to be a useful additional tool in the differential diagnosis of Cushing's syndrome, GRF, a 44 amino-acid residue peptide, seems to be of little value in the diagnosis of active acromegaly. However, both releasing factors are useful in the follow-up of these two hypersecretory diseases after neurosurgical treatment. In addition, the measurement of CRF and GRF activity by radioimmunoassay in the peripheral circulation will be of great importance in making the diagnosis of ectopic CRF- and GRF-secretion, syndromes which have been appreciated only in recent years. Furthermore, CRF and GRF-stimulation tests are useful in elucidating the cause of anterior pituitary failure and GRF may be of benefit for the treatment of pituitary dwarfism. However, it has to be stressed that the results of the new CRF and GRF tests can only be interpreted together with results of other established pituitary function tests and the neuroradiological evaluation of the hypothalamo-pituitary area.

Adrenocorticotropic Hormone↗
PubMed2000

Increased levothyroxine requirements presenting as "inappropriate" TSH secretion syndrome in a patient with nephrotic syndrome.

Patients with primary thyroid failure on levothyroxine (LT4) replacement who develop nephrotic syndrome (NS) may rarely present with an increase in LT4 requirements. In this report, we describe a patient with thyroid failure following radioactive iodine ablation for Graves' disease who required an escalation of LT4 doses following the onset of NS. The case presented with disproportionately elevated TSH levels in the presence of normal (or slightly subnormal) thyroid hormone levels, thus, masquerading as a state of "inappropriate" TSH secretion. This pattern of extreme dysregulation in thyroid function indices due to urinary loss of thyroid hormones has not been previously described in NS, and, therefore, extends the spectrum of endocrine manifestations of NS.

Adult↗
PubMed2006

[Treatment of pituitary gland hyperfunction: from acromegaly to prolactinoma].

Evidence based drug therapy is currently available for the treatment of prolactinomas and growth hormone secreting adenomas (acromegaly). Dopamine agonists such as bromocriptine, quinagolide or cabergoline represent the standard therapy for the treatment of micro- and macro-prolaktinomas. In pregnancy, more differentiated, individual and patient-adapted therapeutic procedures have to be considered. Transsphenoidal adenomectomy is the treatment of choice for patients suffering from acromegaly. If biochemical cure (defined by normalized IGF-1 serum levels or by a GH nadir <1 microg/l during a 3-h oral glucose tolerance test) cannot be achieved, somatostatin analogues such as octreotide and lanreotide are effective. In some cases, dopamine agonists can be added. In therapy-resistant cases, growth hormone receptor antagonists can be used.

Acromegaly↗
PubMed2000

Intracavernous sinus ectopic adrenocorticotropin-secreting tumours causing therapeutic failure in transsphenoidal surgery for Cushing's disease.

BACKGROUND: The major cause of surgical failure in the treatment of Cushing's disease lies in unsuccessful identification of adenomas. In the present study, we investigated the pathogenesis of negative exploration in transsphenoidal surgery for Cushing's disease by analyzing neuro-imaging studies, endocrinological examination and selective venous sampling. METHODS: Thirty patients with ACTH-dependent Cushing's syndrome that met the endocrinological criteria for Cushing's disease were treated by transsphenoidal microsurgery. Depending on positive or negative identification of adenomas during the surgery, selective adenomectomy, partial hypophysectomy or subtotal hypophysectomy was performed. FINDINGS: All nine patients who underwent selective adenomectomy showed endocrinological remission and did not need any hormone replacement therapy. Of the 12 patients who underwent partial hypophysectomy, 11 showed normalization of hypercortisolism but seven needed permanent replacement of hydrocortisone. Four patients underwent subtotal hypophysectomy because no adenoma could be identified in spite of detailed exploration, and three of them showed remission but with permanent requirements of hydrocortisone and other pituitary hormones. Five patients, whose hypercortisolism persisted after initial surgery, underwent total hypophysectomy as secondary transsphenoidal surgery, and only one of them showed endocrinological remission. Of the six patients with surgical failure, four were thought to be rare cases in whom the ACTH-secreting tumours may have occurred ectopically in the cavernous sinus without direct contact with the pituitary gland. Endocrinologically, these four patients showed a low or no response to corticotropin releasing hormone (CRH) stimulation, and for three of them, radiation therapy to the sellar region including the cavernous sinus was effective for persistent hypercortisolemia. INTERPRETATION: Recognition of an ectopic intracavernous sinus ACTH-secreting tumour as a pathological entity for Cushing's disease may not only enhance the diagnostic accuracy but also be important for determining the optimal surgical mode for persistent Cushing's disease.

Adenoma↗
PubMed2000

The value of intraoperative cytology during transsphenoidal surgery for ACTH-secreting microadenoma.

BACKGROUND: Microsurgical selective adenomectomy is the best established method available for the treatment of Cushing's disease. However, this surgical method warrants still more efforts to improve the results in minute microadenomas. In this paper the authors evaluate a method of intraoperative cytological investigations during transsphenoidal surgery. METHOD: Between January 1997 and September 1999, transsphenoidal surgery was performed in 75 patients with the diagnosis of Cushing's disease. Fifty-one cases of proven microadenomas were reviewed. FINDINGS: Of 51 cases, 33 tumors were 3 mm or less in diameter, here after called minute adenomas. In 49 of 51 (96.1%) microadenomas, adenoma tissue was identified by intraoperative cytological techniques. Postoperatively, only 35 of 51 ACTH-secreting microadenomas (68.6%) were confirmed by immunostaining methods. This lower percentage was most probably due to the small amount of tissue obtained. Therefore, in 14 cases (including 12 minute adenomas) the presence of the adenoma was only proven by cytological preparation and clinical outcome. The sensitivity of cytological preparations in cases of confirmed Cushing's disease was 100%. INTERPRETATION: The method described here was particularly well suited for the intraoperative discrimination and documentation of minute adenomas. Cytological preparation appears to be effective in improving the adenoma finding rate and the surgical outcome in cases of Cushing's disease.

Adenoma↗
PubMed1986

Dominant inheritance of the complex of myxomas, spotty pigmentation, and endocrine overactivity.

We describe a family in which lentigines were present in the index patient, in three of her seven siblings, in their mother, and in a niece (the daughter of an affected sister). Cutaneous myxomas were present in the index patient, in two of her brothers, and probably in their mother. In addition, the index patient had two cardiac myxomas. multiple myxoid mammary fibroadenomas, and the Cushing syndrome, and an affected brother had acromegaly caused by a growth hormone-secreting tumor of the pituitary gland. Thus, at least one manifestation of the complex of myxomas, spotty pigmentation, and endocrine overactivity has occurred in three successive generations of this family. Both male and female family members were affected, and 5 of the 11 children of affected persons had the disorder. The karyotypes of two affected persons were normal. These observations are consistent with mendelian dominant inheritance of the syndrome.

Adolescent↗