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)

Bilateral, simultaneous inferior petrosal sinus blood-sampling for determinations of ACTH levels has improved the ability to establish a differential diagnosis of Cushing's disease, particularly in patients whose endocrinological studies show equivocal results and whose computed tomography scans yield negative or inconclusive findings. Individual anatomical variations in the configuration of the sinus and insignificant differences between the ACTH levels obtained from its two sides may be a problem. Seven patients with clinically and biochemically typical Cushing's disease and one with atypical Cushing's disease were examined. An alternative approach for catheterization of the inferior petrosal sinus through the deep vertebral venous plexus was used in those patients who did not have direct connections between the sinus and the internal jugular vein. In two of the eight patients, only ACTH secretion stimulated with corticotrophin-releasing factor could unmask a significant difference between the ACTH levels on the two sides which was not obvious from the basal ACTH levels. Beta-lipotrophin values showed a behaviour similar to that of ACTH values, but because of a slower turn-over rate, the CRF-induced peaks were better visible. The aetiology of Cushing's disease was established from the results of this examination and corresponded with the surgical findings in this series, with the exception of one in which the patient had a highly atypical form of the disease. The abnormal ACTH source was found later to be a thymus carcinoid.
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PMID:Corticotrophin-releasing factor-test used with bilateral, simultaneous inferior petrosal sinus blood-sampling for the diagnosis of pituitary-dependent Cushing's disease. 282 Jun 18

A carcinoid tumour presenting as Cushing's syndrome is reported. Although no tumour mass could be initially identified the patient returned with first a liver and subsequently a cerebellar mass both of which were resected. Only at post-mortem was the lung primary discovered. ACTH, gastrin-releasing peptide (GRP) and calcitonin gene-related peptide were elevated in plasma before resection of the hepatic tumour. These peptides were demonstrated in both the hepatic and cerebellar tumours by immunocytochemistry and radioimmunoassay. This case illustrates the occasional tendency of primary lung carcinoids to remain small and clinically undetectable while generating secondary tumours which are symptomatic. It is suggested that immunological demonstration of GRP may be diagnostically helpful in directing attention to the lung as a primary site in neuroendocrine tumours which present in this fashion.
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PMID:Co-secretion of calcitonin gene-related peptide, gastrin-releasing peptide and ACTH by a carcinoid tumour metastasizing to the cerebellum. 282 44

A 26-yr-old male was submitted to bilateral adrenalectomy in 1977 for Cushing's syndrome. Some months later he developed intense skin hyperpigmentation together with increased ACTH levels (149 to 4000 ng/l). The sellar region was always normal in X-ray studies. In April 1985, when the patient complained of chest pain, a chest x-ray showed a polycyclic mass in the upper left lobe of the lung. ACTH ranged from 20,000 to 100,000 ng/l, with no response to CRF or cyproheptadine administration. Urinary 5-OH-indolacetic acid was negative. Thoracotomy was performed in July 1985 with resection of two intrapulmonary masses. Histologic study demonstrated a carcinoid tumor, with positive neuron-specific enolase and ACTH immunochemical stain. ACTH concentration in tumoral tissue was 91 pg/g tissue. After surgery ACTH fell dramatically to 37 ng/l, and has remained at this level since then, associated with resolution of the skin hyperpigmentation.
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PMID:Diagnosis of lung carcinoid with cutaneous hyperpigmentation eight years after bilateral adrenalectomy. 283 Dec 64

The use of a standard CRF test, using 100 micrograms i.v. synthetic ovine CRF-41, has been assessed in 22 patients with surgically-proven Cushing's disease and one patient with the ectopic ACTH syndrome secondary to a bronchial carcinoid. Three of the 22 patients, and the single patient with the ectopic ACTH syndrome, failed to produce a rise in serum cortisol to above the normal range in response to CRF-41. However, 18/22 patients with Cushing's disease had an enhanced cortisol response to CRF, including four patients who were resistant to high-dose dexamethasone. One patient with Cushing's disease responded to CRF-41 with an excessive cortisol response on one occasion, and a rise within the normal range on a second. Ten patients with simple obesity (mean weight 101 kg) were given CRF 0.5 microgram/kg, and their results compared to a control group of seven normal weight females (mean weight 58 kg). Peak serum cortisol responses to CRF were significantly less than in the control group, even when the dose was increased to 1 microgram/kg in six of the obese patients. Peak plasma ACTH responses were not significantly different between control and patient groups. It is concluded that the serum cortisol response to CRF is enhanced in the majority (but not all) of patients with Cushing's disease, and is attenuated in simple obesity. The reason for this attenuation requires further study.
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PMID:The use of CRF-41 in the differential diagnosis of Cushing's syndrome and obesity. 283 93

We report on a case of ectopic Cushing's syndrome due to a thymic carcinoid tumour with periodic hormonogenesis. Periods of hormonal production averaged 27 days. Prior to bilateral adrenalectomy, mean (s.d) values of ACTH and cortisol were 202.1 (50.3) pg/ml and 46 (14.7) micrograms/dl, ACTH rising to 3996 +/- 425 pg/ml (P less than 0.01) and cortisol falling to 6.3 +/- 1.5 micrograms/dl (P less than 0.01) in the immediate postoperative period. During the late postoperative period (2-13 months following surgery) ACTH levels fell to 509.3 (123.8) pg/ml (P less than 0.01), but remaining even higher (P less than 0.01) than before adrenalectomy. The pattern of ACTH in the present case suggests the existence of a negative feedback exerted by the cortisol over tumoral ACTH.
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PMID:Ectopic Cushing's syndrome with periodic hormonogenesis--a case suggesting a pathogenetic mechanism. 283 33

Cushing's syndrome due to bronchial carcinoid tumors that secrete adrenocorticotropin (ACTH) may be difficult to distinguish from pituitary Cushing's disease, since the responses to dexamethasone and metyrapone are sometimes similar. Recently, the ACTH and cortisol responses to ovine corticotropin-releasing factor (oCRF) have been shown to be different in pituitary Cushing's disease than in Cushing's syndrome due to other causes. It is not known if the response to oCRF can distinguish pituitary Cushing's disease from those ACTH-secreting bronchial carcinoid tumors that respond to dexamethasone and metyrapone. A case of Cushing's syndrome due to an ACTH-secreting bronchial carcinoid is described in which the responses to dexamethasone, metyrapone, and oCRF were indistinguishable from the responses observed in pituitary Cushing's disease. A bronchial carcinoid tumor should be considered even when responses to dexamethasone, metyrapone, and oCRF suggest pituitary Cushing's disease.
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PMID:Ectopic ACTH syndrome caused by a bronchial carcinoid tumor responsive to dexamethasone, metyrapone, and corticotropin-releasing factor. 284 Aug 23

Two patients with Cushing's syndrome due to lung carcinoid tumours were given the long-acting somatostatin analogue SMS 201-995 (Sandostatin). One received a single 50 micrograms dose which produced a 50% reduction in circulating ACTH levels within 4 h. The other has been maintained in clinical and biochemical remission for 10 weeks on 100 micrograms tid. This is the first report of the successful use of SMS 201-995 in carcinoid-induced Cushing's syndrome, and suggests that this hormone analogue could be valuable in the long term medical management of such patients.
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PMID:Lung carcinoid with Cushing's syndrome: control of serum ACTH and cortisol levels using SMS 201-995 (sandostatin). 284 45

A male patient with corticotropin-releasing factor (CRF) and adrenocorticotropin (ACTH)-producing syndrome is described. Soon after being referred to us the patient developed pneumonia, anaemia, oedema and respiratory distress, and died on the 24th day after admission. Autopsy and histology revealed that he had a rare type of multiple endocrine neoplasia (type 1 + paraganglioma) with a mediastinal paraganglioma, parathyroidal hyperplasia, pancreatic islet cell adenoma, duodenal multiple carcinoid tumours and adrenocortical nodular hyperplasia. It was not possible to examine the pituitary. The paraganglioma contained a large amount of immunoreactive (IR)-CRF (606 ng/g wet weight), IR-ACTH (59.4 ng/g wet weight), IR-human proopiomelanocortin n-terminal (1-76) peptide (hNT, 156.8 ng/g wet weight) and IR-beta-lipotropin (beta-LPH, 146.9 ng/g wet weight). The major IR-ACTH, beta-LPH and IR-hNT were eluted at ACTH-(1-39), beta-LPH and hNT marker positions, respectively. Big ACTH was not detected. IR-CRF eluted at the human CRF marker position on Sephadex G-75 chromatography and high performance liquid chromatography (HPLC). The IR-CRF fraction from the HPLC showed CRF bioactivity which paralleled that of synthetic human CRF in monolayer cultured rat anterior pituitary cells. Our results suggest that not only ACTH but CRF produced by the paraganglioma was responsible for the patient's Cushing's syndrome.
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PMID:Multiple endocrine neoplasia with Cushing's syndrome due to paraganglioma producing corticotropin-releasing factor and adrenocorticotropin. 287 33

Carcinoids occur in association with MEN types 1 and 2. To determine the relationship between carcinoids and MEN, we reviewed nine patients with carcinoids and other endocrine tumors. Analyzing these 9 patients and 56 other patients previously described in the literature, we found several clinically important relationships. In contrast to the usual midgut and hindgut origin, most carcinoids associated with MEN (69 percent) are of foregut origin (thymus 24 percent, bronchus 27 percent, stomach 3 percent, and duodenum 14 percent). Carcinoids are more commonly associated with MEN type 1 than MEN type 2 (59 patients and 6 patients, respectively). Thymic carcinoids associated with MEN are more common in men (15 versus 2), and most (82 percent) are malignant. Bronchial carcinoids associated with MEN are more common in women (15 versus 4), and most (74 percent) are benign. There is a strong association between thymic carcinoids and parathyroid tumors and between bronchial carcinoids and pituitary tumors. Most patients with carcinoids and hyperparathyroidism (82 percent) have had parathyroid hyperplasia or multiple parathyroid adenomas. Thus, carcinoids may occur in association with both MEN type I and MEN type II. MEN should be suspected in patients with foregut carcinoids. Patients with MEN and ectopic ACTH production should be considered to have bronchial carcinoids if they are female and thymic carcinoid if they are male. The thymus should be routinely removed in patients with MEN type I because of the possible presence of an ectopic parathyroid gland in this tissue and to prevent subsequent development of a carcinoid tumor.
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PMID:Carcinoids associated with multiple endocrine neoplasia syndromes. 288 72

Four patients with advanced endocrine malignancies were treated with a somatostatin analogue (SMS 201-995) for palliation of hormone-induced symptoms during 3-6 months. Two had the carcinoid syndrome (one midgut and one foregut), one had medullary thyroid carcinoma and an ectopic ACTH syndrome, and one patient had a metastatic gastrinoma. The carcinoid patients had excellent symptomatic relief with a low dose of the drug, 50 micrograms subcutaneously twice daily, in one case despite progression of tumour disease and biochemical tumour markers. These findings indicate an action of the drug not only on hormonal release but also at peripheral sites. The patient with medullary thyroid carcinoma had relief of gastrointestinal symptoms when the drug dose was increased (100 micrograms twice daily). The levels of ACTH in peripheral blood were reduced, but not the calcitonin levels. The gastrinoma patient had undergone a major pancreatic resection (Whipple procedure) and was treated with omeprazole. SMS 201-995 reduced the peripheral gastrin levels acutely, but during the treatment fasting gastrin values increased, and the tumour growth progressed. Treatment was stopped owing to elevated fasting glucose level, increased steatorrhoea, and clinical attacks of cholangitis. Special attention is advocated for patients with major pancreatic resection and biliary reconstruction, who may be susceptible to physiological effects of somatostatin (or its analogues)--that is, impaired insulin release and decreased motility.
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PMID:The use of a long-acting somatostatin analogue in the treatment of advanced endocrine malignancies with gastrointestinal symptoms. 289 Nov 86


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