Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P61278 (somatostatin)
 22,083 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thyrotropic pituitary adenoma is a rare disease fortunately diagnosed with increasing frequency. Its originality lies in the fact that it occurs in two very different pathological contexts. The first form raises few problems: the occurrence of a pituitary tumoral syndrome in a patient with hypothyroidism, even of relatively short duration, should suggest the diagnosis. In such cases of reactive thyrotropic adenoma, simple correction of the hypothyroidism by replacement therapy seems to be capable not only of curing the thyroid disorder and lowering TSH values, but also of promoting regression of the pituitary tumour. Primary autonomous adenoma responsible for TSH hypersecretion followed by thyrotoxicosis belongs to the realm of inappropriate TSH secretion, and it should be diagnosed whenever high, or even normal (at least non-suppressed). TSH levels coexist with peripheral hyperthyroidism. As a rule, the presence of a pituitary tumour differentiates this adenoma from non-tumoral inappropriate TSH secretion due to resistance to thyroid hormones. Primary thyrotropic pituitary adenoma is treated by surgery. In case of failure or relapse, radiotherapy or medical treatment with a dopaminergic agonist or a long-acting somatostatin analogue may be considered.
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PMID:[Thyrotropic pituitary adenoma. Clinical aspects, course and treatment]. 295 27

Cystic dystrophy of the duodenal wall developing in heterotopic pancreas is a rare disease. Weight loss and painless vomiting due to duodenal stenosis where the main clinical manifestations of this entity in a chronic alcoholic patient. Diagnosis was made by using an ultrasonic-endoscope equipped with a miniprobe. Although surgical treatment is usually recommended in this situation, the clinical condition of this patient improved dramatically after subcutaneous injections of somatostatin analog (octreotide). This treatment was maintained during 9 months and no recurrence was observed during the follow-up period.
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PMID:[Treatment with octreotide of stenosing cystic dystrophy on heterotopic pancreas of the duodenal wall]. 888 76

Carcinoid syndrome is a relatively rare disease, generally associated with poor prognosis. Conventional diagnostic and therapeutic methods often prove inadequate and ineffective. New therapeutic options have recently been provided by Radiolabeled long-acting somatostatin analogs (Octreotide), alpha interferon, 131 MIBG and non-pharmacological methods--embolization of the hepatic artery, gene therapy, and combined therapies.
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PMID:New therapeutic options of carcinoid syndrome metastatic to the liver. 1221 46

Cystic dystrophy of the duodenal wall in heterotopic pancreas is a rare disease affecting younger men in particular. On account of its difficult diagnosis and nonspecific signs, it was first described in 1970. A 51-year-old male patient was admitted for clarification of several months of severe pain in the upper abdomen accompanied by weight-loss, and due to ultrasonically established suspicion of carcinoma in the head of the pancreas. Clinical examination and laboratory tests were nonsignificant. Barium meal and esophagogastroduodenoscopy revealed a severe deformation of the duodenum. Biopsy of the duodenal mucosa detected moderate inflammatory changes. Ultrasound examination showed cystic changes in the duodenal wall and in the vicinity of the head of the pancreas. Only with the aid of endoscopic ultrasound, could bizarre cystic changes in the submucosa of the duodenal wall be detected, which was also confirmed by computer tomography. The signs of disease are nonspecific. Duodenal biopsy also does not reveal its cause since the typical lesions lie deeper in the submucosa or muscularis propria. Cystic changes with fibrosis, pancreatic ducts and lobi without anatomic or vascular connection to the pancreas are found. The anomaly involves, above all, the stomach and duodenum. Prior to the introduction of imaging techniques such as endoscopic ultrasonography, computer tomography and nuclear magnetic resonance, the diagnosis could only be made by means of an operative procedure. Treatment is primarily surgical; smaller cysts can be treated by endoscopic fenestration. Pharmacological treatment with somatostatin is rarely performed.
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PMID:Cystic dystrophy of the duodenal wall in a heterotopic pancreas. 1263 71

Zollinger-Ellison syndrome is characterised by refractory peptic ulcer disease, severe diarrhoea and gastric acid hypersecretion associated with an islet-cell tumour of the pancreas (gastrinoma). The true incidence and prevalence of this rare disease is unknown; in the US, the frequency is one per one million people and the age at presentation varies from 7 to 90 years. Zollinger-Ellison syndrome is sporadic in 62-80% of cases and in 20-38% of cases is associated with multiple endocrine neoplasia type 1 (MEN 1). The diagnosis of Zollinger-Ellison syndrome is certain when the plasma gastrin is >1000 pg/mL and the basal acid output is >15 mEq/h in patients with an intact stomach, >5 mEq/h in gastrectomised patients, or when this hypergastrinemia is associated with a pH <2. The treatment is based on control of gastric acid hypersecretion and of the malignant tumour and its possible metastases. Proton pump inhibitors are the most effective antisecretory drugs and can be administered in the elderly at high dosages without drug-related adverse effects. As an initial therapy, daily dosages of omeprazole 80-100 mg or pantoprazole 40-160 mg are employed. In long-term treatment the doses can be greatly reduced once effective control of the gastric output has been established. Intravenous proton pump inhibitors may be administered when patients cannot take oral therapy, particularly in acute conditions. All sporadic localised gastrinomas should be excised if possible. When liver metastases are also present, their debulking may improve symptoms and survival, and facilitate medical treatment. There is some controversy as to the surgical approach for gastrinomas associated with MEN 1. Somatostatin analogues can be useful in reducing gastric acid hypersecretion, serum gastrin and gastric enterochromaffin-like (ECL) cells and can thus contribute to treating the disease more effectively. Their antiproliferative effect can be used in treating liver metastases. Chemotherapy is not the therapy of choice in patients with gastrinomas and is indicated only in those with malignant progressive disease; interferon alpha, embolisation and chemoembolisation are not advisable for the elderly. The treatment of elderly Zollinger-Ellison syndrome patients, similarly to all elderly oncological patients, should be based on the use of comprehensive geriatric assessment. This will enable the clinician to define the functional status of the elderly person, to decide whether the patient can tolerate surgery and/or the stress of antineoplastic therapy, and finally, to determine whether this patient can tolerate an aggressive treatment for Zollinger-Ellison syndrome or whether the only possible choice is palliative relief of symptoms.
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PMID:Optimal treatment of Zollinger-Ellison syndrome and related conditions in elderly patients. 1465 42

Carcinoid tumors derived from neuroendocrine cells can release serotonin and other vasoactive substances into the systemic circulation, resulting in carcinoid syndrome. Testicular carcinoid, a rare disease accounting for less than 1% of all testicular neoplasms, rarely manifests symptoms of carcinoid syndrome. We describe a case of carcinoid syndrome arising from a primary testicular carcinoid tumor. A 21-year-old male patient presented with facial flushing and diarrhea for 5 years. He had an enlarged left testis and a 1-cm, ill-defined, hard, non-tender mass in his right testis. His 24 h urinary excretion of 5-hydroxyindoleacetic acid was elevated (16.1 mg/day). Somatostatin receptor scintigraphy correlated with carcinoid tumor in both testes. Following bilateral orchiectomy, the patient's facial flushing and diarrhea disappeared.
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PMID:Primary carcinoid tumor of the bilateral testis associated with carcinoid syndrome. 1550 16

The prevalence of malignant pheochromocytoma is about 10%, and is somewhat higher for paraganglioma. A problem for clinical follow-up is that patients with "benign" histopathologic findings may develop metastatic disease. At the first international symposium on pheochromocytoma in Bethesda (2005) experts from different disciplines and patients shared their experiences, and the present knowledge of this rare disease was updated. The discussion related to future strategies for better clinical/histopathologic diagnosis and understanding of different geno- and phenotypes. Curative surgery can only seldom be performed because of multiple metastases. The main therapeutic goal is therefore often tumor reduction and control of hypertension. To date the best adjunct to surgery is radionuclide therapy using 131I-MIBG, but the background information for optimal treatment is still incomplete. Certain patients may benefit from 131I-MIBG combined with radiotherapy via somatostatin receptors expressed by the tumor, or the combination with chemotherapy. The need for future multicenter studies was emphasized. In experimental models the work on enhanced expression of amine transporters critical for radiotherapy is continued. Ongoing microarray studies will reveal novel intracellular pathways of importance for proliferation/cell cycle control, which can be inhibited by pharmacologic tools.
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PMID:Malignant pheochromocytoma: state of the field with future projections. 1710 14

Central hyperthyroidism due to a thyrotropin (TSH)-secreting pituitary adenoma is a rare cause of hyperthyroidism, representing 0.5-1.0% of all pituitary adenomas. The etiopathogenesis of TSH-secreting-adenomas is unknown and no definite role for various oncogenes has been proven. Patients with TSH-secreting adenoma usually present with signs and symptoms of hyperthyroidism milder than those in patients with hyperthyroidism of thyroid origin, in addition to symptoms secondary to mass effects of the pituitary tumour. Mixed pituitary tumours co-secrete growth hormone and prolactin. The characteristic biochemical abnormalities are normal or high serum TSH concentrations in the presence of elevated total and/or free thyroid hormones concentrations. Measurement of markers of peripheral thyroid hormone action and dynamic tests may aid in the differential diagnosis with the syndrome of resistance to thyroid hormone. Neuroimaging is fundamental to visualize the pituitary tumor. Therapy of TSH-secreting adenomas can be accomplished by surgery, radiation therapies, and medical treatment with somatostatin analogs or dopamine agonists. Nowadays, and in contrast with the first reports on this rare disease, most patients are well controlled by current therapies.
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PMID:Thyrotropin-secreting pituitary adenomas: biological and molecular features, diagnosis and therapy. 1892 69

Acromegaly is a rare disease usually caused by growth hormone (GH) hypersecretion, due to a pituitary adenoma; in very rare cases, acromegaly is due to ectopic secretion of GHRH, responsible for pituitary hyperplasia. Owing to its insidious onset, acromegaly is often diagnosed late (4 to > 10 years after onset), at an average age of about 40 years, in front of an acquired, slowly progressing disfigurement mainly involving the face and extremities. Acromegaly has also rheumatologic, cardiovascular, respiratory and metabolic consequences which determine its prognosis. The diagnosis is based on an increased serum GH concentration unsuppressed following an oral glucose load (oral glucose tolerance test -OGTT-) and an increased insulin-like growth factor-I (IGF-I); according to a 2000 Consensus statement, if the basal serum GH is above 0,4microg/L (1.2mIU/L) and/or if the IGF-I is elevated, an OGTT must be performed. If the lowest GH value (nadir) during OGTT remains above 1microg/L (3mIU/L), acromegaly is confirmed. With the generalized use of very sensitive assays nowadays, it has recently been considered that this cutoff should be decreased to 0,3microg/L (0.9mIU/L). Treatment is aimed at correcting (or preventing) tumor compression by excising the culprit lesion, and at reducing GH and IGF-I levels to normal values (or at least to a "safe" GH level of < 2microg/L or < 6mIU/L). A stepwise therapeutic strategy is used: transsphenoidal surgery is often the first-line treatment; when surgery fails to correct GH/IGF-I hypersecretion, medical treatment with somatostatin analogs and/or radiotherapy can be used, somatostatin analogs being generally preferred; the GH antagonist (pegvisomant) is used in patients that are resistant or intolerant to somatostatin analogs. Prognosis of acromegaly has improved in the recent years: adequate hormonal disease control is achieved in most cases, allowing life expectancy similar to that of the general population.
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PMID:[Acromegaly]. 1900 12

Pheochromocytoma (PCC) is a rare disease, mainly sporadic, but also associated with some familial disorders, with a malignancy frequency of approximately 10%. Only the presence of distant metastases, derived from large pleomorphic chromaffin cells, is widely accepted as a criterion of malignancy. Variable symptoms may be caused by production and release of catecholamines. Since there is no curative treatment for malignant PCC and due to its unfavorable prognosis, assuring quality of life is one of the main therapeutic objectives. Besides a long-term medical treatment of symptoms using selective alpha-1 blockers and nonselective, noncompetitive alpha- and/or beta-blockers, debulking surgery is the first treatment step. In case of a sufficient uptake of (123)I-MIBG treatment with targeted radiation therapy, use of (131)I-MIBG is an option as an adjuvant therapy, following debulking surgery. Chemotherapy should be applied to patients without positive MIBG-scan, with no response to (131)I-MIBG or progression after radionuclide treatment, and especially in cases with high proliferation index. The most effective chemotherapy regimen appears to be the CVD-scheme, including cyclophosphamide, vincristine, and dacarbazine. The so-called targeted molecular therapies with treatment combinations of temozolomide and thalidomide, or sunitinib monotherapy, and novel therapeutic somatostatin analogues have shown promising results and should thus encourage clinical trials to improve the prognosis of metastatic PCC. Within this review the current treatment modalities and novel molecular strategies in the management of this disease are discussed and a treatment algorithm is suggested.
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PMID:Treatment of malignant pheochromocytoma. 1967 13


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