Pathophysiology and clinical aspects of the ectopic GH‐releasing hormone syndrome

Losa, Marco; Werder, Klaus von

doi:10.1046/j.1365-2265.1997.2311048.x

Cited by 59 publications

(50 citation statements)

References 70 publications

(189 reference statements)

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“…In the rare situation where a non-pituitary aetiology is suspected, the serum GHRH level should be measured; GHRH secretion is responsible for 0.5% of all cases (Losa & Von Werder 1997).…”

Section: Diagnosismentioning

confidence: 99%

60 YEARS OF NEUROENDOCRINOLOGY: Acromegaly

Căpățînă¹,

Wass²

2015

Journal of Endocrinology

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Acromegaly (ACM) is a chronic, progressive disorder caused by the persistent hypersecretion of GH, in the vast majority of cases secreted by a pituitary adenoma. The consequent increase in IGF1 (a GH-induced liver protein) is responsible for most clinical features and for the systemic complications associated with increased mortality. The clinical diagnosis, based on symptoms related to GH excess or the presence of a pituitary mass, is often delayed many years because of the slow progression of the disease. Initial testing relies on measuring the serum IGF1 concentration. The oral glucose tolerance test with concomitant GH measurement is the gold-standard diagnostic test. The therapeutic options for ACM are surgery, medical treatment, and radiotherapy (RT). The outcome of surgery is very good for microadenomas (80-90% cure rate), but at least half of the macroadenomas (most frequently encountered in ACM patients) are not cured surgically. Somatostatin analogs are mainly indicated after surgical failure. Currently their routine use as primary therapy is not recommended. Dopamine agonists are useful in a minority of cases. Pegvisomant is indicated for patients refractory to surgery and other medical treatments. RT is employed sparingly, in cases of persistent disease activity despite other treatments, due to its long-term side effects. With complex, combined treatment, at least three-quarters of the cases are controlled according to current criteria. With proper control of the disease, the specific complications are partially improved and the mortality rate is close to that of the background population.

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“…In the rare situation where a non-pituitary aetiology is suspected, the serum GHRH level should be measured; GHRH secretion is responsible for 0.5% of all cases (Losa & Von Werder 1997).…”

Section: Diagnosismentioning

confidence: 99%

60 YEARS OF NEUROENDOCRINOLOGY: Acromegaly

Căpățînă¹,

Wass²

2015

Journal of Endocrinology

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“…The indolent course of bronchial carcinoids led to delay in the diagnosis of patients with a mean of 10.6 years as opposed to pancreatic endocrine tumors, where the mean duration for the diagnosis was around five years [108][109]. The classic manifestations of patients with ectopic GHRH producing tumors were found to be similar to those found in GH secreting pituitary adenomas.…”

Section: Ectopic Ghrh Productionmentioning

confidence: 92%

“…Although plasma GHRH higher than 300 ng/liter is usually reported in patients with ectopic GHRH, however three cases of acromegaly due to ectopic secretion of GHRH have been reported with plasma GHRH between 100 and 300 ng/liter [109]. The decrease in GHRH level upon tumor resection would further prove ectopic GHRH secretion however in many cases metastasis is present at the time of the diagnosis and surgery would not be a valid option [108][109]. From the registry of the sole laboratory in France which measures plasma GHRH assays, 21 patients with ectopic GHRH secretion presenting with acromegaly were identified between 1983 and 2008.…”

Section: Ectopic Ghrh Productionmentioning

confidence: 98%

“…In 1984 it was discovered that the main biological activity of GHRH lies in its first 29 amino acids [107]. It was also found, that those GHRH which were extracted from tumor tissue, had the same amino acid sequence as the GHRH produced from the hypothalamus [108]. GHRH acts through binding to its receptor on somatotroph cells, hence activating adenylate cyclase resulting in an increase in intracellular cyclic AMP.…”

Section: Ectopic Ghrh Productionmentioning

confidence: 99%

“…The decrease in GHRH level upon tumor resection would further prove ectopic GHRH secretion however in many cases metastasis is present at the time of the diagnosis and surgery would not be a valid option [108][109]. From the registry of the sole laboratory in France which measures plasma GHRH assays, 21 patients with ectopic GHRH secretion presenting with acromegaly were identified between 1983 and 2008.…”

Section: /12mentioning

confidence: 99%

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Endocrine Paraneoplastic Syndromes: A Review

Arabi¹

2015

EMIJ

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It is important for the endocrinologist and oncologist to recognize these paraneoplastic endocrine syndromes since this may help in defining the cancer type and hence lead to the appropriate management. In addition, these syndromes are a major cause of morbidity and mortality. Moreover, AbstractParaneoplastic endocrine syndromes result from ectopic production of hormones by different tumors. Hypercalcemia of malignancy is the most common, mostly caused by ectopic parathyroid hormone related peptide (PTHrP) production which increases bone resorption. Other causes include the rare ectopic parathyroid hormone (PTH) production, ectopic production of 1, 25-(OH)2 vitamin D by the tumor and its adjacent macrophages and bone metastasis which by itself in addition to the local production of PTHrP at the level of the bone lead to bone resorption and thus hypercalcemia. Treatment includes extracellular fluid volume repletion, bisphosphonates or denosumab and calcitonin. Ectopic Cushing's syndrome caused by ectopic ACTH production results in hypokalemia, proximal muscle weakness, easy bruisability, hypertension, diabetes and psychiatric abnormalities including depression and mood disorders. Different diagnostic measures help to differentiate Cushing's disease from ectopic Cushing's syndrome. Treatment includes surgical resection of tumor and medical therapy to suppress excess cortisol production. Ectopic secretion of ADH has been associated with different tumor types. The best treatment options include removal of the underlying tumor, chemotherapy, or radiotherapy in addition to free water restriction, demeclocycline and vaptans. Ectopic growth hormone releasing hormone secretion has been identified with carcinoids, phechromocytoma and other tumors. Surgery is the mainstay of therapy with systemic therapy used in metastatic disease. Other rare ectopic hormone syndromes have also been identified including ectopic prolactin, ectopic erythropoietin, and thrombopoetin in addition to others. Keywords 2/12paraneoplastic endocrine syndromes may be the first presenting feature of malignancy [2,3]. This paper provides an overview on the common PNES, their prevalence, diagnosis, prognosis and response to treatment (Table 1). Methodology of Literature SearchEligible studies were identified by a search, from year 1920 up till February 2014, in Medline, PUBMED and EMBASE using a refined search strategy with several terms used including "endocrine paraneoplastic syndromes", "ectopic hormones", "humoral hypercalcemia of malignancy", "ectopic parathyroid hormone related peptide", "ectopic ACTH", "ectopic Cushing syndrome", "ectopic antidiuretic hormone", "syndrome of inappropriate ADH", "ectopic growth hormone releasing hormone", "ectopic growth hormone", "ectopic prolactin", "ectopic thyroid stimulating hormone" in addition to several others. In addition, searches of personal collections and the reference lists of eligible studies were identified with their full text papers being retrieved. A-Hypercalcemia of MalignancyHistorical Perspec...

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Normal and Abnormal Growth

Walvoord¹,

Eugster²

2002

Developmental Endocrinology

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Pathophysiology and clinical aspects of the ectopic GH‐releasing hormone syndrome

Cited by 59 publications

References 70 publications

60 YEARS OF NEUROENDOCRINOLOGY: Acromegaly

60 YEARS OF NEUROENDOCRINOLOGY: Acromegaly

Endocrine Paraneoplastic Syndromes: A Review

Normal and Abnormal Growth

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