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

Cushing's disease developed in a 5-year-old girl with acute lymphoblastic leukemia 18 months after her last therapeutic exposure to adrenal glucocorticosteroids. Obesity, hyperpigmentation, striae, osteoporosis, and hirsutism were accompanied by elevated levels of plasma cortisol. These showed no diurnal fluctuation and they were not suppressed by dexamethasone. At autopsy, the adrenal glands were enlarged and the pituitary gland showed increased numbers of basophils of the adrenocorticotropic hormone (ACTH)/melanocyte-stimulating hormone secreting type. Leukemic infiltrates in brain tissue were prominent in the hypothalamus and in the limbic system. It is postulated that the destructive leukemic infiltrate of the limbic system removed a restraining influence on pituitary function, with basophilic hyperplasia, ACTH hypersecretion, adrenocortical hypertrophy, and clinical Cushing's disease the consequences.
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PMID:Cushing's syndrome and acute lymphoblastic leukemia. 27 79

With an antiserum against human beta-endorphin (beta-EP) crossreacting less than 2% with human beta-lipotropin (beta-LPH) by weight we have developed a radioimmunoassay that can detect 1 pg beta-EP in diluted raw plasma. In a.m. fasting plasma of 14 normal subjects beta-EP ranged from less than 5 to 45 pg/ml. beta-EP was elevated in untreated, but normal in successfully treated Cushing's disease; undetectable in a patient with adrenal adenoma; extremely high in Nelson's syndrome; and elevated in a patient with bronchogenic carcinoma before, but undetectable after tumor resection. In subjects with intact hypothalamic-pituitary-adrenal axis, beta-EP was undetectable after dexamethasone and increased after metyrapone administration and insulin-induced hypoglycemia. beta-EP concentration was considerably lower in serum than in simultaneously collected plasma, but increased in serum left unfrozen for several hours after clot removal. Thus, beta-EP behaves like a hormone responding to the same stimuli as ACTH and beta-LPH and blood appears to contain enzymes both generating and destroying immunoreactive beta-EP.
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PMID:Specific radioimmunoassay of human beta-endorphin in unextracted plasma. 46 83

Transsphenoidal microsurgery was performed in four patients with a pituitary microadenoma who presented Cushing's disease. Preoperative features were the following: 1) Clinical and laboratory signs of hypercortisolism 2) No radiological evidence of an adrenal tumor 3) Normal or increased ACTH plasma levels 4) Sellar tomograms suggesting the presence of a pituitary microadenoma in only one case. Postoperative outcome after 3 to 30 months follow-up showed persistence or even improved reactivity of the various pituitary functions and especially the early occurence of corticotropin deficiency in all 4 patients. This isolated deficiency regressed spontaneously after 6 and 12 months (2/4 cases). Twenty-four hour studies of ACTH and cortisol profiles and dexamethasone suppression as well as response to metyrapone confirmed the resumption of physiological corticotropin regulation.
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PMID:[Pituitary microadenoma of Cushing's disease. Course following transsphenoidal excision. Four cases (author's transl)]. 50 29

Mineralocorticoid function of the adrenal cortex and the activity of the renin-angiotensin system were studied in patients with Itsenko-Cushing's disease before (36 cases) and after (27 cases) the treatment with chloditan--an adrenocortical inhibitor. Minertalocorticoid function of the adrenal cortex was assessed by the blood immunoreactive aldosterone content, urinary 18-aldosterone-glucuronide excretion, and the rate of aldosterone secretion. Condition of the renin-angiotensin system was assesed by the blood renin activity. Aldosterone and renin concentration was studied by the radioimmunological method. There proved to be a reverse relationship between the blood aldosterone concentraiton and the severity of Itsenko-Cushing's disease. Elevation of the mineralocorticoid function of the adrenal cortex in Itsenko-Cushing's disease was due to activation of the renin angiotensin system. During clinical remission of the disease following a course of chloditan treatment the patient displayed an increased blood aldosterone level and a rise of its secretion. The minralocorticoid function of the adrenal cortex was intensified at the state of clinical remission because of an increased blood corticotropin concentration.
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PMID:[Mineralocorticoid function of the adrenal cortex and the renin--angiotensin system in Itsenko-Cushing's disease]. 70 55

A sensitive and specific radioimmunoassay for human beta-lipotropin (betah-LPH) in unextracted plasma was developed using pur betah-LPH as tracer and standard and an antiserum not cross-reacting with human beta-MSH and hACTH. In healthy volunteers plasma betah-LPH ranged from less than 20 to 150 pg/ml at 8:00 a.m. and rose after metyrapone administration. betah-LPH was very low in panhypopituitarism, normal in most patients with untreated Cushing's disease, elevated in acromegaly and extremely high in Nelson's syndrome.
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PMID:Radioimmunoassay of human beta-lipotropin in unextracted plasma. 92 35

A case is presented of generalized skin hyperpigmentation due to alpha-MSH hypersecretion from the pituitary that was most marked in the light-exposed areas. The patient also had secondary adrenal dysfunction, peripheral lymphadenopathy, streptococcal glomerulonephritis and malabsorption. Analysis of this patient's alpha-MSH using high-pressure liquid chromatography (HPLC) showed a novel acetylation profile compared to normal individuals and to patients with Cushing's disease and Nelson's syndrome. Glucocorticoid replacement therapy resulted in suppression of alpha-MSH hypersecretion and complete resolution of the illness.
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PMID:A case of skin hyperpigmentation due to alpha-MSH hypersecretion. 131 79

Transgenic mice that developed adrenocorticotropic hormone (ACTH)-producing pituitary tumors were generated with the polyoma early region promotor linked to a cDNA encoding polyoma large T antigen (PyLT). Light microscopic examination of the pituitaries showed normal morphology at 4 months of age, either unremarkable morphology or microadenoma formation at 9 months of age, and up to 5 mm large adenomas in clinically ill transgenic mice at 13-16 months of age. At age 9 months, transgenic mice weighed significantly more than corresponding control mice, but they began wasting at approximately 1 year of age. The adrenal glands of these older PyLT-1 mice showed a weight increase and exhibited a medullary hyperplasia. Subcutaneous transplants of transgenic pituitary tumors to nontransgenic, immunocompetent mice resulted in tumors with a morphology and ACTH immunoreactivity similar to the primary tumor. The effects of hypercorticotropism were more enhanced and occurred with a shorter latency in the mice carrying transgene pituitary transplants than in the PyLT-1 transgenic mice themselves. Moreover, these transplanted mice showed a weight increase with an axial deposition pattern and hypertrophy of the adrenal cortex that resembled the findings in human Cushing's disease. Plasma ACTH levels were significantly increased in clinically ill transgenic mice and even higher levels were found in the transplant mice. Thus, both murine models should be useful for studying Cushing's disease.
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PMID:Transgenic mice that develop pituitary tumors. A model for Cushing's disease. 131 82

It has been hypothesized by Lamberts and coworkers in their analysis of 15 cases that adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas may be derived from either the anterior lobe or the intermediate lobe. The intermediate lobe type of Cushing's disease is thought to be controlled through a hypothalamic pathway and is characterized by hyperprolactinemia; suppressibility of cortisol with bromocriptine, and lower sensitivity to dexamethasone. The authors investigated the validity of this hypothesis in 125 cases of ACTH-secreting pituitary microadenomas by analyzing the endocrine findings, the locations of the microadenomas, and alpha-melanocyte stimulating hormone (alpha-MSH) immunoreactivity in the adenoma cells. No significant differences in the basal hormone levels, cortisol suppressibility with bromocriptine, sensitivity to dexamethasone, and recurrence rate were observed between patients with the microadenoma adjacent to the posterior lobe (considered typical of the intermediate lobe-derived tumor) or those with the microadenoma located in the anterior lobe. The locations of the microadenoma were not correlated with alpha-MSH immunoreactivity in the adenoma cells. No significant differences in endocrine findings were noticed between adenomas positive or negative for alpha-MSH. Thus, Cushing's disease cannot be simply divided into either the anterior lobe type or the intermediate lobe type by endocrinological evaluation as described by Lamberts, et al.
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PMID:Endocrinological evaluation of ACTH-secreting pituitary microadenomas: their location and alpha-melanocyte stimulating hormone immunoreactivity. 131 55

Recent reports suggest that, contrary to radioimmunoassays (RIA), immunoradiometric assays (IRMA) artifactually decrease plasma ACTH levels in patients with the ectopic ACTH syndrome. Discrepancies between RIA and IRMA results may provide a means of discriminating this entity from Cushing's disease. We have compared the results of these two techniques, together with those of a beta-endorphin assay, in 17 patients with Cushing's disease, 9 with the ectopic ACTH syndrome and 30 controls. ACTH-RIA and ACTH-IRMA levels in patients with Cushing's disease were similar (17.5 +/- 2.5 vs 15.1 +/- 2.8 pmol/l) and were correlated (rs = 0.59, p less than 0.01). ACTH-RIA levels in patients with the ectopic ACTH syndrome were higher than ACTH-IRMA levels (27.3 +/- 2.9 vs 14.5 +/- 2.5, p less than 0.01) and these did not correlate. The ACTH-RIA and ACTH-RIA/ACTH-IRMA ratio levels in patients with the ectopic ACTH syndrome were higher than those of patients with Cushing's disease (p less than 0.01), but they overlapped with these in 27 and 31% of cases respectively. Plasma beta-endorphin level was higher in patients with the ectopic ACTH syndrome than in patients with Cushing's disease (81.9 +/- 19.4 vs 26.4 +/- 5.6 pmol/l, p less than 0.01) and was correlated with ACTH only in patients with Cushing's disease. The overlap in beta-endorphin and beta-endorphin/ACTH-IRMA molar ratio levels between the two groups were 19 and 27% respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Comparative value of plasma ACTH and beta-endorphin measurement with three different commercial kits for the etiological diagnosis of ACTH-dependent Cushing's syndrome. 131 30

Procedures were carried out in a 12-year-old girl to relate Ewing's sarcoma of the left tibia with Cushing's syndrome. Computed tomography revealed a normal pituitary and hypothalamus but bilateral adrenal hyperplasia without focal enlargement, thus readily excluding hypothalamic-pituitary-adrenal tumor. Negative results from a high-dose dexamethasone suppression test do not support pituitary-dependent Cushing's disease. Ewing's sarcoma was diagnosed on tibial biopsy. The regression of the physical and biochemical findings of Cushing's syndrome subsequent to amputation of the left lower leg strongly suggests ectopic Cushing's syndrome caused by Ewing's sarcoma. Immunohistochemical studies of the resected bone were negative for corticotropin but positive for corticotropin releasing factor-like peptide. We conclude that this is the first reported case of ectopic Cushing's syndrome in a child that is caused by Ewing's sarcoma secreting corticotropin releasing factor-like peptide.
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PMID:Cushing's syndrome caused by Ewing's sarcoma secreting corticotropin releasing factor-like peptide. 132 12


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