Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0007095 (carcinoid)
 6,990 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The adenylate cyclase responses of the human GH or ACTH producing pituitary adenomas and ectopic ACTH producing tumors to TRH, LH-RH, biogenic amines, peptides hormones, PGE1 and rat median eminence extract (MEE) have been examined. Out of 4 GH producing pituitary adenomas obtained from patients with active acromegaly at hypophysectomy two were stimulated by TRH, two by LH-RH, three by norepinephrine, one by dopamine, four by PGE1 and none by serotonin. Glucagon stimulated the adenylate cyclase in one of three and MEE in both of two tested. The positive responses of paradoxical GH release after TRH and/or LH-RH before surgery in these patients coincidentally related to the response of adenylate cyclase of each pituitary adenoma. There seems, however, to be no consistent correlation between the adenylate cyclase responses to biogenic amines and the GH release after L-Dopa or 5-hydroxytroptophan tested. The adenylate cyclase of a pituitary adenoma from case of Cushing's disease was stimulated by LH-RH, norepinephrine glucagon and MEE but not by TRH. Plasma levels of ACTH, beta-MSH and cortisol increased after LH-RH but not after TRH in this patient before hypophysectomy. The adenylate cyclase of two ectopic ACTH producing tumors (gastric carcinoid and malignant thymoma) was activated by TRH, LH-RH, norepinephrine, epinephrine, serotonin, PGE1 and MEE. These results indicate the presence of multiple hormone receptors in GH or ACTH producing pituitary adenomas and ectopic ACTH producing tumors, and suggest that the paradoxical GH or ACTH release after TRH and/or LH-RH injection in acromegaly and Cushing's syndrome might be caused by an alteration of the cellular membrane receptors of the pituitary adenomas.
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PMID:Adenylate cyclase of GH and ACTH producing tumors of human: activation by non-specific hormones and other bioactive substances. 19 Feb 56

A 14-year-old Japanese male with a previously undescribed combination of bilateral pheochromocytoma and an islet cell tumor of the pancreas is presented. The combination of endocrine neoplasms in this patient overlaps multiple endocrine neoplasia (MEN) Type 1 and Type 2. A total of 14 reported cases of MEN overlapping Type 1 and Type 2 is reviewed. Of the 14, 7 patients with acromegaly developed a paraganglioma(s), 2 patients with Sipple syndrome had a pituitary adenoma, and in the other 5 patients, an intestinal carcinoid or a pancreatic islet-cell tumor occurred in association with either a thyroid medullary carcinoma or a paraganglioma(s). We believe that the occurrence of MEN overlapping Type 1 and Type 2 is more than a fortuitous association, and can be explained on the basis of the neuroectodermal origin.
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PMID:Coexistence of bilateral pheochromocytoma and pancreatic islet cell tumor: report of a case and review of the literature. 21 1

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.
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PMID:[Catheterization of the inferior petrosal sinus. Its role in the diagnosis of Cushing's syndrome. Experience with 23 explorations]. 133 72

In addition to the common presentations of the multiple endocrine neoplasia (MEN) syndromes, unusual organ involvement as rare manifestations of a single disease may occur. Among our patients we have identified four cases in which unusual features of MEN were present. In the first patient, bilateral adrenal cortical adenoma, parathyroid adenoma, multiple pancreatic tumors, and follicular thyroid carcinoma were observed. The second patient suffered from thymic carcinoid, parathyroid hyperplasia, gastrinoma, and pituitary adenoma. Additionally, one family was discovered in which medullary thyroid carcinoma (MTC), Hirschsprung's disease, and pheochromocytoma occurred and another family had MTC and ovarian cancer. Based on these observations, we stress the importance of screening for MEN syndromes in all patients with pathologic findings in any endocrine organ.
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PMID:Unusual features of multiple endocrine neoplasia. 136 16

Acromegaly is a unique condition characterized by chronic growth hormone (GH) and insulin-like growth factor-1 (IGF-1) hypersecretion usually due to a pituitary adenoma. Rarely, acromegaly can result from a GH-releasing hormone carcinoid or pancreatic neoplasm which stimulates the normal pituitary to secrete GH. This review describes the interactions between acromegaly and the gastrointestinal system. In contrast to the soft tissue and skeletal changes, clinical organomegaly of the liver, kidney, and spleen is unusual in patients with acromegaly and should warrant further investigations. The prevalence of cholelithiasis is notably increased by the use of the otherwise effective GH-lowering somatostatin analog, octreotide. Patients on long-term therapy with this agent may require anticholelithogenic treatment. The frequency of malignant and premalignant polyps of the colon justify the routine screening for these lesions in newly diagnosed patients with acromegaly.
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PMID:Hepatobiliary and gastrointestinal manifestations of acromegaly. 161 13

Ectopic GHRH is a relatively uncommon cause of acromegaly, which should be differentiated from pituitary adenoma, in order to avoid damage to the pituitary gland from unnecessary interventions. We report here on a 66-year-old man with acromegaly due to a GHRH-secreting bronchial carcinoid tumour, who recovered completely following removal of the tumour. His hormonal status was studied before and after the operation. Basal GH, GHRH, IGF-I and PRL levels, as well as plasma GH response to glucose load and TRH administration were abnormal before the operation, and became normal thereafter. The somatostatin analogue SMS 201-995 was found to be a potent inhibitor of the ectopic GHRH and the GH secretion (greater than 500 to 42 ng/l and 15.4 micrograms/l to 0.8 microgram/l, respectively). The effect on GHRH proved to be due to direct effect of somatostatin on the tumour cells, as demonstrated in tissue culture studies. A mixed meal was found immediately to suppress GHRH levels without such an effect on GH secretion. We conclude that the neuroendocrine tests usually practised in acromegaly cannot differentiate between ectopic GHRH secretion and pituitary adenoma. High plasma GHRH levels may serve as a diagnostic test for excessive GHRH production, which is almost always ectopic. These high levels are suppressible by somatostatin and a mixed meal.
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PMID:Acromegaly due to ectopic growth hormone-releasing hormone secretion by a bronchial carcinoid tumour. Dynamic hormonal responses to various stimuli. 168 1

The molecular forms of ACTH secreted by established human small cell lung cancer (SCLC) cells and primary cultures derived from a bronchial carcinoid tumour, a pituitary adenoma and hyperplastic pituitary tissue have been characterized by Sephadex G-75 chromatography and quantified with two novel immunoradiometric assays for ACTH and ACTH precursor peptides. Pro-opiomelanocortin (POMC; Mr 31,000) and pro-ACTH (Mr 22,000) were secreted by all cell types. No smaller peptides were identified in the culture media from SCLC and bronchial carcinoid cells, implying a deficiency in the enzymes and/or intracellular organelles required for extensive POMC processing. A more heterogeneous profile of ACTH-containing peptides was produced by cells of pituitary origin, indicating more extensive proteolytic processing of POMC. However, the major peptide secreted by cells from a large aggressive pituitary adenoma was unprocessed POMC (Mr 31,000). These results suggest that both lung and pituitary cells in vitro retain their in-vivo pattern of POMC processing and provide valuable models in which to study the regulation of ACTH synthesis and secretion.
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PMID:Comparison of ACTH and ACTH precursor peptides secreted by human pituitary and lung tumour cells in vitro. 215 69

A 35-year male patient with clinical and biochemical manifestations of severe hypercorticism was thought to have Cushing's syndrome of pituitary origin. However, the surgically removed pituitary adenoma was not confirmed by pathological examination. There was no improvement after transsphenoidal microsurgery Chest CT scan showed a small mass located at the upper-lobe of the right lung. This tumor (1.8 x 1.0 x 1.0 cm) was removed and one course of radiotherapy with linear accelerator was given. Remission was achieved clinically and biochemically after these therapies. The diagnosis of bronchial carcinoid was confirmed by pathological findings. Metastasis of a lymph node was also proved. The tumor cells was found to contain ACTH and related peptides with radioimmunoassay, immunocytochemistry and electron microscopy. Gel filtration of the tumor extracts showed molecular heterogeniety of ACTH related peptides and that the big-form were in large percentage.
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PMID:[Ectopic ACTH syndrome caused by bronchial carcinoid]. 256 Oct 95

Apudomas are neoplasms known to produce many biologically active amines. Some of these tumors have been known for 70 years, but over the last 15 years a wealth of new knowledge has been obtained. This study reports experience with apudomas over an 8-year period (1975-1983) at The Piedmont Hospital. There were 159 tumors with the potential for producing various ectopic hormones as well as biologically active amines, including 112 oat cell carcinomas, 37 carcinoid tumors, 4 paragangliomas, 2 pancreatic islet cell tumors, 2 medullary thyroid carcinomas, 1 pituitary adenoma, and 1 pinealocytoma. Of the 112 oat cell carcinomas, only 2 per cent had hormone-related symptoms. Of the carcinoid tumors, 12 were found in the appendix, 9 in the small intestine, 9 in the lungs, 4 in the colon and 2 in the rectum. Nineteen of the 37 carcinoids had hormone-related symptoms but none had a classic carcinoid syndrome. A third group of apudomas included several of relative rarity, the paragangliomas, the medullary thyroid carcinomas, the pancreatic islet cell tumors, pituitary adenomas, and pinealocytomas. Of this latter group, only the pancreatic islet cell tumors had symptomatology that could be related to ectopic hormone production.
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PMID:Apudomas. 282 Feb 86

A young female patient, with clinical and biochemical manifestations of severe hypercorticism and with the presence of a pituitary adenoma shown by computerized tomography, was thought to have Cushing's syndrome of hypophysial origin. However, the surgically-removed pituitary adenoma contained no ACTH, by immunocytology, and hypercorticism persisted after transsphenoidal adenomectomy. The patient died and autopsy demonstrated an ACTH and corticotrophin releasing factor (CRF)-containing bronchial carcinoid. It can be concluded that bronchial carcinoids can produce ACTH and CRF and can mimic the clinical and biochemical manifestations of pituitary Cushing's syndrome. Thus, the localization of the primary site of hypercorticism can be extremely difficult in patients who have an insidious, occult extrapituitary tumour. Further work is required to establish whether CRF plays a role in the causation of Cushing's syndrome and whether the simultaneous secretion of this peptide can modify the clinical and biochemical manifestations of the ectopic ACTH syndrome.
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PMID:ACTH and CRF-producing bronchial carcinoid associated with Cushing's syndrome. 302 67


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