[Medical topics: treatment of hyperpituitarism; stomach cancer after gastric resection].
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A total of 818 impotent men were evaluated in a multidisciplinary Sexual Function Center for reproductive hormone levels. Data are presented demonstrating an overall rate of endocrinopathy at 19.3 percent. Various age-dependent trends in the incidence of abnormal levels are noted. Hypogonadism as well as hyperpituitarism appear to increase in incidence with increasing age. Hyperprolactinemia, however, seems to be relatively constant in occurrence. Because of the likelihood of detecting endocrine abnormalities, a recommendation is made for routine hormonal screening of all patients complaining of impotence.
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.
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.
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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.
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Hyperplasia of the parathyroid glands is a central feature of familial multiple endocrine neoplasia type 1. We used cultured bovine parathyroid cells to test for mitogenic activity in plasma from patients with this disorder. Normal plasma stimulated [3H]thymidine incorporation, on the average, to the same extent as it was stimulated in a plasma-free control culture. This contrasted with the results of the tests with plasma from patients with familial multiple endocrine neoplasia type 1, in which parathyroid mitogenic activity increased 2400 percent over the control value (P less than 0.001). Plasma from these patients also stimulated the proliferation of bovine parathyroid cells in culture, whereas plasma from normal subjects inhibited it. Parathyroid mitogenic activity in plasma from the patients with familial multiple endocrine neoplasia type 1 was greater than that in plasma from patients with various other disorders, including sporadic primary hyperparathyroidism (with adenoma, hyperplasia, or cancer of the parathyroid), sporadic primary hypergastrinemia, sporadic pituitary tumor, familial hypocalciuric hypercalcemia, and multiple endocrine neoplasia type 2 (P less than 0.05). Parathyroid mitogenic activity in the plasma of patients with familial multiple endocrine neoplasia type 1 persisted for up to four years after total parathyroidectomy. The plasma also had far more mitogenic activity in cultures of parathyroid cells than did optimal concentrations of known growth factors or of any parathyroid secretagogue. This mitogenic activity had an apparent molecular weight of 50,000 to 55,000. We conclude that primary hyperparathyroidism in familial multiple endocrine neoplasia type 1 may have a humoral cause.
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Forty-five patients with acromegaly or gigantism were reviewed for musculoskeletal abnormalities. Abnormalities of peripheral joints occurred in 74% of the patients and spinal involvement in 47%, leading to significant morbidity. Joint abnormalities most frequently affected the large joints (hips, knees and shoulders) but the wrist and hand were also involved. The radiological features of acromegalic arthropathy are described, including vertical widening of the hip joint, enthesopathy and osteophytosis. A favourable response to treatment is associated with a less severe arthropathy and a good functional outcome.