Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P01189 (beta-endorphin)
 21,003 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 4-year-old girl had abdominal distention, muscular weakness, renal tubular dysfunction, and hypertension associated with hypokalemic metabolic alkalosis. There were no clinical symptoms of cortisol deficiency, but there was excessive deoxycorticosterone and cortisocsterone production. Basal plasma aldosterone levels were undetectable; however, adrenocorticotropic hormone (ACTH) stimulation brought plasma aldosterone levels up to normal. The urinary pregnanediol, tetrahydro-deoxycorticosterone (THDOC), and tetrahydrocorticosterone (THB) concentrations were elevated. Stimulation of ACTH failed to increase urinary 17-ketosteroid, 17-hydroxycorticosteroid, or plasma cortisol levels significantly, while urinary THDOC, THB, and plasma corticosterone concentrations were further elevated. The elevated plasma corticosteroid intermediates were suppressed by dexamethasone administration. When physiologic doses of dexamethasone were administered, the hypertension, electrolyte imbalance, and abnormal corticosteroid secretion were all corrected. The studies indicated a partial 17alpha-hydroxylase defect in this patient.
 ...
PMID:Hypokalemic crisis simulating intestinal obstruction in a 4-year-old girl. A consequence of 17alpha-hydroxylase deficiency. 97 20

A 7-year-old spayed female Cocker Spaniel was hospitalized with a history of chronic vomiting, anorexia, and weight loss. Laboratory abnormalities included leukocytosis, metabolic alkalosis, hypoglycemia, hypoproteinemia, and hyperinsulinemia. Gastroscopy and ultrasonography revealed multiple gastric masses and a possible pancreatic mass, respectively. Examination of tissues obtained at necropsy showed a pancreatic adenocarcinoma with hepatic metastasis, gastric hypertrophy, and multiple duodenal ulcers. Immunocytochemical staining of the neoplasia was positive for pancreatic polypeptide (PP) and insulin and negative for gastrin, calcitonin, adrenocorticotropic hormone (ACTH), serotonin, L-enkephalin, chromagranin, glucagon, and somatostatin. Subsequent serum gastrin and PP assays showed a fasting hypergastrinemia with a normal response of gastrin to provocative testing and extremely increased PP values. The high PP values may have resulted in the vomiting and gastrointestinal ulceration. A PP-secreting tumor has not previously been reported in the dog.
 ...
PMID:Pancreatic polypeptide and insulin-secreting tumor in a dog with duodenal ulcers and hypertrophic gastritis. 267 25

A patient with a left lower lung mass had muscle weakness, generalized hyperpigmentation, metabolic alkalosis, and profound hypokalemia. His elevated serum cortisol, corticosterone, and adrenocorticotropic hormone (ACTH) concentrations were not suppressed after midnight dexamethasone administration. Light and electron microscopic sections of the lung mass fitted the pathological criteria for adenocarcinoma. Immunocytochemical analysis of the tumor demonstrated specific staining with antibody to beta-endorphin, suggesting that the tumor cells made the common precursor molecule of ACTH, beta-lipotropin, and endorphin. This is, to the best of our knowledge, only the second case report of pulmonary adenocarcinoma associated with the syndrome of ectopic ACTH.
 ...
PMID:Adenocarcinoma of the lung causing ectopic adrenocorticotropic hormone syndrome. 709 52

A 57-year-old white man presented with metabolic alkalosis, hypokalemia (pH 7.58, HCO3 >50 mEq/L, serum K 1.8 mEq/L) and hypertension. The initial evaluation was significant for markedly elevated serum cortisol and adrenocorticotropic hormone (ACTH) level; neither hormone showed circadian rhythm or suppression with high-dose dexamethasone. Perihilar and supraclavicular masses were found to consist of undifferentiated small cell carcinoma. Ectopic ACTH syndrome was diagnosed. In spite of progressively rising hormone levels (ACTH, 723 pg/dL; and cortisol, 212 microgram/dL), his severe metabolic alkalosis was largely corrected by aggressive treatment with potassium chloride alone. Possible mechanisms of these clinical findings are discussed.
 ...
PMID:Correction of metabolic alkalosis by potassium chloride in ectopic adrenocorticotropic hormone syndrome. 884 Sep 54

A 68-year-old patient with severe hypokalemia and metabolic alkalosis is described. Six years before admission he had been treated for a small cell bronchial carcinoma. We discuss the diagnostic approach of hypokalemia and the way in which we reached the diagnosis. The patient suffered from metastatic small cell carcinoma with a very high plasma adrenocorticotropic hormone concentration, possibly due to production of corticotropin-releasing hormone by the malignancy.
 ...
PMID:Sixty-eight-year-old patient with hypokalemia. 1124 16

Ectopic adrenocorticotropic hormone (ACTH) and/or corticotropin-releasing hormone (CRH) are associated with a growing list of tumors. We report a 69-year-old white man with a history of high-grade prostate carcinoma and widely metastatic adenocarcinoma who presented with metabolic alkalosis, hypokalemia, and hypertension secondary to ectopic ACTH and CRH secretion. Laboratory values were consistent with hypokalemia and metabolic alkalosis. Markedly elevated serum cortisol (135 microg/dL), ACTH (1,387 pg/dL), CRH (69 pg/dL), and urine free cortisol (16,276 microg/24 h) levels were found. Chest computed tomographic (CT) scan showed small noncalcified parenchymal densities; however, bronchoscopy and bronchoalveolar lavage washings were unremarkable for a neoplastic process. Abdominal CT scan and magnetic resonance imaging showed multiple small liver lesions and multiple thoracic and lumbar intensities consistent with diffuse metastatic disease. Histological analysis of a biopsy specimen from the thoracic spine showed an undifferentiated adenocarcinoma consistent with a prostate primary tumor. The severe metabolic alkalosis secondary to glucocorticoid-induced excessive mineralocorticoid activity was treated with potassium supplements, spironolactone, and ketoconazole. In this case report, we describe an unusual tumor associated with ectopic ACTH and CRH production and the pharmacodynamic relationship of plasma cortisol levels and urinary cortisol excretion with ketoconazole treatment.
 ...
PMID:Hypokalemia, metabolic alkalosis, and hypertension: Cushing's syndrome in a patient with metastatic prostate adenocarcinoma. 1127 85

Ectopic Cushing's syndrome due to various malignancies is not uncommon. However, a few cases of ectopic Cushing's syndrome caused by corticotropin-releasing hormone (CRH), or CRH with adrenocorticotropic hormone (ACTH) have been reported. A 28-year-old woman presented with acute upper gastrointestinal bleeding caused by an active ulcer, located atypically in the 2nd portion of duodenum. Further work-up revealed high gastrin levels and abdominal computed tomography (CT) scans showed a large pancreatic head mass with multiple liver metastases. The serum cortisol and ACTH levels were checked due to hypokalemia with metabolic alkalosis and recent amenorrhea. Cortisol and ACTH were both highly elevated with pituitary hyperplasia and elevated CRH. The existence of ectopic ACTH and CRH in the liver biopsy was also demonstrated immunohistochemically. Since an operation was not feasible, chemotherapy was conducted using paclitaxel and etoposide. These two drugs were chosen according to the IN VITRO chemotherapy response assay to maximize the treatment. This report demonstrates concurrent ACTH- and CRH-related ectopic Cushing's syndrome caused by malignant gastrinoma with multiple liver metastases that was treated with marginal success using a multidisciplinary medical approach.
 ...
PMID:Ectopic Cushing's syndrome due to concurrent corticotropin-releasing hormone (CRH) and adrenocorticotropic hormone (ACTH) secreted by malignant gastrinoma. 1728 28

Although ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a well-known paraneoplastic phenomenon, an association with large-cell neuroendocrine carcinoma of the lung (LCNEC) has not been reported. We describe a 63-year-old man with metastatic LCNEC to the left temporomandibular joint (TMJ) who presented with progressive muscle weakness and bilateral lower leg edema for 2 weeks. He did not have a typical Cushingoid appearance nor used diuretics. His newly noted hypertension, hypokalemia (plasma potassium (K) concentration 1.8 mEq/L) with renal K wasting, and metabolic alkalosis suggested a state of mineralocorticoid excess. His plasma renin activity and aldosterone concentrations were low, but cortisol and ACTH levels were extremely elevated, consistent with ACTH-dependent Cushing's syndrome. Nonsuppressible plasma cortisol level and normal sella turcica on magnetic resonance imaging pointed to EAS. A strongly positive stain for ACTH from the metastatic left TMJ mass supported LCNEC-related EAS. His hypokalemia and hypertension were controlled with spironolactone and K supplementation. This is the first reported case of EAS in LCNEC and should be kept in mind as a cause of hypokalemia in lung cancer patients.
 ...
PMID:Ectopic ACTH syndrome associated with large-cell neuroendocrine carcinoma of the lung. 1809 71

A 79-year-old woman, a smoker, presented to the emergency department with a 2-week history of progressive dyspnoea and peripheral oedema, preceded by flu-like symptoms that had failed to improve despite antibiotics. Examination identified severe hypertension and signs consistent with severe fluid overload. Baseline chest X ray showed a right-sided abnormality. In the context of an extensive smoking history CT thorax was indicated which demonstrated a right-sided hilar mass and lymphadenopathy. Blood tests showed an unexplained metabolic alkalosis with partial respiratory compensation and hypokalaemia prompting endocrinological tests investigating Cushing's syndrome. Urinary cortisol was measured at 3174 nmol/L/24 h (normal<560), serum adrenocorticotropic hormone (ACTH) of 215 ng/L (normal<46). Pleural fluid cytology confirmed a diagnosis of small-cell lung cancer (SCLC). The patient's condition deteriorated despite intravenous diuretics and nitrates, metyrapone and non-invasive ventilation. Treatment was withdrawn 1 week after admission. The clinical course in the presence of biochemical derangement and SCLC is highly suggestive of paraneoplastic ectopic ACTH secretion.
...
PMID:Cardio-respiratory failure secondary to ectopic Cushing's syndrome as the index presentation of small-cell lung cancer. 2394 25

Small cell lung cancer (SCLC), which originated from neuroendocrine tissue, can develop into paraneoplastic endocrine syndromes, such as Cushing syndrome, because of an inappropriate secretion of ectopic adrenocorticotropic hormone (ACTH). This paraneoplastic syndrome is known to be a poor prognostic factor in SCLC. The reason for poor survival may be because of a higher risk of infection associated with hypercortisolemia. Therefore, early detection and appropriate treatment for this syndrome is necessary. But the diagnosis is challenging and the source of ACTH production can be difficult to identify. We report a 69-year-old male patient who had severe hypokalemia, metabolic alkalosis, and hypertension as manifestations of an ACTH-secreting small cell carcinoma of the lung. He was treated with ketoconazole and spironolactone to control the ACTH dependent Cushing syndrome. He survived for 15 months after chemotherapy, which is unusual considering the poor outcome of the ectopic ATH syndrome associated with SCLC.
...
PMID:A Case of Ectopic Adrenocorticotropic Hormone Syndrome in Small Cell Lung Cancer. 2650 41


1 2 Next >>