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)

We examined the long-term effects of octreotide, a somatostatin analogue, on ventilation in a case of cirrhotic hypoxemia. After daily administration of octreotide for one month, the dyspnea on exertion was notably ameliorated, although pulmonary gas exchange was only slightly improved. The octreotide therapy reduced the hypoxic ventilatory drive, which may be one reason for the relief of the dyspnea sensation.
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PMID:Effect of octreotide on ventilation and dyspnea sensation in a patient with cirrhotic hypoxemia. 764 10

Dilated cardiomyopathy associated with acromegaly is rare, but may improve with octreotide, a somatostatin analogue. The authors give the first description here of paradoxical worsening in cardiac function during such treatment, with the onset of episodes of acute decompensation following each attempt at starting treatment. Thus worsening was confirmed objectively by a challenge test with octreotide: increased dyspnea, fall in shortening fraction and in echocardiographic cardiac output (of 17 to 14% and 4 to 3 l/min respectively), a decrease in isotopic ejection fraction from 15 to 6% and this in parallel with efficacy regarding hormone levels of GH and IGF1 and a reduction in tumour size by CT scan. No further episode of decompensation occurred after treatment was stopped permanently. The patient underwent a transplant 3 months later. Suppression of the positive inotropic effect of GH by octreotide, associated with an increase in peripheral resistance is suggested. A negative inotropic effect of this hormonal analogue on too advanced a case of heart disease is also a possibility.
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PMID:[Dilated cardiomyopathy in acromegaly worsening under octreotide treatment. Apropos of a case]. 849 2

Pleural effusion is a rare complication of chronic pancreatitis. We report a case observed in a 39-year-old patient hospitalized for dyspnea and pain in the lower left thorax. Chest x-ray revealed left pleural effusion. The exudative pleural fluid contained a very high amylase level. CT-scan revealed a pseudo-cyst of the tail of the pancreas extending into contact with the diaphragm and the chronic pancreatitis lesions. Medical treatment associating thoracic drainage, somatostatin analog, and antibiotics was unsuccessful. Thoracoscopic pleural decortication was performed. The patient then developed pneumonia involving the left base. A new antibiotic regimen was delivered and the pseudocyst was drained percutaneously under CT guidance. The clinical course was favorable at six months with partial involution of the pseudocyst and regression of the pulmonary images.
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PMID:[A rare complication of chronic pancreatitis: pancreatico-pleural fistula]. 1469 97

A 59-year-old woman complained of increasing pain in the left abdomen and of diarrheal symptoms after left-sided retroperitoneoscopic living donor nephrectomy. Computed tomography revealed chyloretroperitoneum. The fluid was drained percutaneously, followed by recurrent drain replacement surgery. Her diet was changed to short-chain and medium-chain fatty acids without success. After onset of dyspnea due to chylothorax, the donor underwent thoracic drainage, fully parenteral therapy, and finally somatostatin application therapy. The postoperative management of severe chylous fistula is difficult. Therefore, we recommend an early start of maximal conservative therapy. Surgical options depend on pain occurrence or mechanical problems.
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PMID:Chyloretroperitoneum with secondary chylothorax after retroperitoneoscopic donor nephrectomy. 1623 Jan 68

Pancreaticopleural fistula is an uncommon clinical entity that appears in patients with a history of chronic pancreatitis. Apropos of the case of a 40-year-old man in our hospital, we reviewed the literature and found 40 publications with little more than 100 patients. All of these publications highlight the atypical presentation with dyspnea as the first symptom and stress that diagnosis is based on magnetic resonance pancreatography. Endoscopic retrograde cholangiopancreatography is used in confusing cases and in planning the optimal surgical approach. Initial treatment should consist of conservative medical therapy, including total parenteral nutrition and somatostatin or its analogues. If the fistula persists or recurs, various options are available such as surgery, placement of a transpapillary pancreatic stent or nasopancreatic tube. The overall mortality rate from pancreaticopleural fistula is approximately 5%.
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PMID:[Pancreaticopleural fistula: diagnostic tests and treatment]. 1642 Sep 51

Hypothalamic GHRH is secreted into the portal system, binds to specific surface receptors of the somatotroph cell and elicits intracellular signals that modulate pituitary GH synthesis and/or secretion. Moreover, GHRH is synthesized and expressed in multiple extrapituitary tissues. Excessive peripheral production of GHRH by a tumor source would therefore be expected to cause somatotroph cell hyperstimulation, increased GH secretion and eventually pituitary acromegaly. Immunoreactive GHRH is present in several tumors, including carcinoid tumors, pancreatic cell tumors, small cell lung cancers, endometrial tumors, adrenal adenomas, and pheochromocytomas which have been reported to secrete GHRH. Acromegaly in these patients, however, is uncommon. The distinction of pituitary vs. extrapituitary acromegaly is extremely important in planning effective management. Regardless of the cause, GH and IGF-1 are invariably elevated and GH levels fail to suppress (<1 microg/l) after an oral glucose load in all forms of acromegaly. Dynamic pituitary tests are not helpful in distinguishing acromegalic patients with pituitary tumors from those harbouring extrapituitary tumors. Plasma GHRH levels are usually elevated in patients with peripheral GHRH-secreting tumors, and are normal or low in patients with pituitary acromegaly. Unique and unexpected clinical features in an acromegalic patient, including respiratory wheezing or dyspnea, facial flushing, peptic ulcers, or renal stones sometimes are helpful in alerting the physician to diagnosing non pituitary endocrine tumors. If no facility to measure plasma GHRH is available, and in the absence of MRI evidence of pituitary adenoma, a CT scan of the thorax and abdominal ultrasound could be performed to exclude with good approximation the possibility of an ectopic GHRH syndrome. Surgical resection of the tumor secreting ectopic GHRH should be the logical approach to a patient with ectopic GHRH syndrome. Standard chemotherapy directed at GHRH-producing carcinoid tumors is generally unsuccessful in controlling the activated GH axis. Somatostatin analogs provide an effective option for medical management of carcinoid patients, especially those with recurrent disease. In fact, long-acting somatostatin analogs may be able to control not only the ectopic hormonal secretion syndrome, but also, in some instances, tumor growth. Therefore, although cytotoxic chemotherapy, pituitary surgery, or irradiation still remain available therapeutic options, long-acting somatostatin analogs are now preferred as a second-line therapy in patients with carcinoid tumors and ectopic GHRH-syndrome.
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PMID:Neuroendocrine tumors secreting growth hormone-releasing hormone: Pathophysiological and clinical aspects. 1703 95

A 72-year-old woman visited a clinic for anorexia and general fatigue but no particular abnormality was detected by routine examination at that time. Thereafter, she experienced gradually increasing dyspnea and chest X ray showed right pleural effusion. Idiopathic chylothorax was diagnosed due to the milky effusion with a high concentration of triglyceride (2618 mg/dl) and no apparent causative disease. Irrespective of treatments including dietary restriction, drainage of the pleural space, and somatostatin injections, her effusion did not reduce. The leakage of lymph fluid from the right posterior mediastinum was identified by lymphatic scintigraphy and she was successfully treated with surgical ligation of the thoracic ducts.
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PMID:[Case of idiopathic chylothorax successfully treated with surgical ligation of thoracic ducts]. 1801 31

While TSH-producing adenoma (TSHoma) is rare, the diagnosis is often delayed because the clinical features are heterogeneous. The patient was a 69-year-old woman who had been referred to the Yachiyo Medical Center in August 2008, because of dyspnea, loss of appetite, weight loss of 10 kg, and diarrhea that lasted 4 years. We diagnosed this patient with pituitary TSH-producing macroadenoma. Thyroid hormone concentration was increasing although the serum TSH level was within a normal range after trans-sphenoidal surgery. We considered that because of enlargement of the thyroid gland due to long-term stimulation by TSH, a low concentration of TSH could stimulate the thyroid gland to produce excess T3 or T4. The somatostatin analogue, octreotide was used to control the TSHoma and serum TSH concentration but not thyroid hormone. The octreotide in combination with thiamazole treatment for 14 months controlled thyroid hormone concentration and decreased the thyroid mass, and ultimately, the thiamazole could be stopped. To date, the use of combination therapy of octreotide with thiamazole in patients with remaining TSH-producing adenoma without Basedow's disease is rare, and we suggest that this treatment is one of the therapeutic means to treat recurrence of TSH-producing adenoma after surgery with progressive complications or large thyroid gland.
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PMID:A case of TSH-producing adenoma treated with octreotide in combination with thiamazole for the control of TSH and thyroid hormones after trans-sphenoidal neurosurgery. 2151 53

Chylothorax results from various causes, such as malignancy, trauma, or infection. POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a multisystemic syndrome that is associated with plasma cell disorder. Pleural effusion is a common manifestation of POEMS syndrome, but the association of POEMS syndrome with chylothorax has not been reported. We report on a 61-year-old female patient who initially presented with dyspnea and bilateral leg edema. Importantly, the patient had normal renal function. Her chest X-ray and computed tomographic imaging showed bilateral pleural effusion, and her chest drainage revealed chylothorax. Detailed examination failed to reveal the definitive cause of the chylothorax. She received several treatments for chylothorax, namely, a low-fat diet or fasting, total parenteral nutrition, a somatostatin analog (octreotide), thoracic duct ligation by video-assisted thoracic surgery, and pleurodesis. However, further examination revealed endocrinopathy, monoclonal plasma cell disorder, peripheral neuropathy, and elevation of the serum level of vascular endothelial growth factor. The patient's condition was consequently diagnosed as POEMS syndrome. Eventually, her chylothorax was controlled by pleurodesis, and she was transferred to another hospital for stem cell transplantation. Herein, we report on the apparent first case of POEMS syndrome with chylothorax. In some cases of idiopathic chylothorax, the underlying primary disease may be latent, such as in the present patient. POEMS syndrome is rare, but this syndrome should be included in the differential diagnosis of chylothorax with unexplained etiology.
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PMID:Chylothorax in POEMS syndrome. 2447 87

Neuroendocrine neoplasms (NENs) comprise a heterogeneous group of tumors derived from multipotent neuroendocrine cells that have the inherent ability to synthesize and secrete a variety of substances (peptides and amines). When these substances are bioactive, NENs can present with a related clinical syndrome (functioning NENs) and/or symptoms of mass effects (functioning and nonfunctioning NENs). NENs differ in their pathogenesis, clinical syndromes produced, aspects of biological behavior and response to certain antitumor treatment. The carcinoid syndrome (CS) is the clinical constellation of secretory diarrhea, flushing, wheezing and dyspnea as a result of serotonin production mainly from small intestinal NENs. Complications of CS are frequent and include extensive mesenteric fibrosis, carcinoid heart disease and the life-threatening carcinoid crisis. A variety of substances (mainly hormones) are produced from NENs originating from the pancreas associated with specific symptoms that can lead to their precise diagnosis. NENs may also secrete substances characteristic of other sites of origin and produce a variety of paraneoplastic syndromes and/or change their secretory status with time. Surgery remains the best option for complete tumor resection and symptom relief. Surgery may also be used when medical treatment fails to obtain control of the symptoms along with cytoreductive techniques. Somatostatin analogs (octreotide and lanreotide) constitute the backbone of medical treatment for the majority of functioning NENs as they can alleviate symptoms, stabilize tumor growth and improve the quality of life. Telotristat etiprate is a novel oral agent that inhibits tryptophan hydroxylase, the key enzyme responsible for serotonin production, and can improve the symptoms of CS. Nonspecific and supportive therapies are also used for refractory cases. In this chapter the clinical features of functioning NENs will be analyzed as well as aspects of their diagnosis based on secretory substances and treatment of the hormonal excess.
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PMID:Clinical Syndromes Related to Gastrointestinal Neuroendocrine Neoplasms. 2630 3


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