Profound amplification of secretory‐burst mass and anomalous regularity of ACTH secretory process in patients with Nelson's syndrome compared with Cushing's disease

Aken, Maarten O. van; Pereira, Alberto M.; Berg, Gerrit van den; Romijn, Johannes A.; Veldhuis, Johannes D.; Roelfsema, Ferdinand

doi:10.1111/j.1365-2265.2004.02052.x

Cited by 27 publications

(6 citation statements)

References 37 publications

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“…Although definitive diagnostic criteria for Nelson’s syndrome are lacking, Barbar, et al proposed a new criteria which included an elevated 0800 h plasma level of ACTH (> 500 ng/L) our patient had high ACTH but less as per Barbar’s criteria because of the fact that ACTH being a volatile molecule disintegrates rapidly, so adequate precautions need to be taken when samples of ACTH are taken (1). The most reliable biochemical feature of Nelson’s syndrome is a marked rise of plasma ACTH, which continues to rise after adrenalectomy, as was seen with our patient (4). Radiological diagnosis of Nelson’s syndrome included enlarging pituitary mass of which MRI is the best imaging modality, detecting tumours as small as 3 mm (5).…”

Section: Discussionsupporting

confidence: 82%

Leksell Gamma Knife : An Effective Non Invasive Treatment for Rare Case of Nelson’s Syndrome

Arshad

Laway

Bhat

et al. 2013

Int J Endocrinol Metab

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Nelson’s syndrome nowadays a rare entity results from an adrenocorticotropin (ACTH)–secreting pituitary adenoma in patients with refractory Cushing's disease after a therapeutic bilateral adrenal gland removal. We report a case of 25 year old female with cushing’s disease who was initially managed with medical treatment, but in view of severe persistent hyper cortisol state was subjected to bilateral adrenalectomy following which she developed Nelson’s syndrome after a gap of six years, which was difficult to diagnose because of limited investigations available. Patient was managed with stereotactic radiosurgery (gamma knife surgery).

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Section: Discussionsupporting

confidence: 82%

Leksell Gamma Knife : An Effective Non Invasive Treatment for Rare Case of Nelson’s Syndrome

Arshad

Laway

Bhat

et al. 2013

Int J Endocrinol Metab

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“…41,77,85,90 When the inhibitory influence of steroids is insufficient for longer periods, as in cases in which replacement of glucocorticoid hormones has not been adequate, pituitary ACTH-producing cells can be stimulated excessively, which in turn can lead to adenomatous transformation of these cells, resulting in NS. 77,111,114 What remains intriguing in the pathophysiology of NS is the lack of responsiveness of adenocorticotroph cells to steroid hormone replacement therapy. Partial resistance to glucocorticoids in a monoclonal pituitary adenoma has been found in corticotroph adenomas.…”

Section: Pathophysiology Of the Diseasementioning

confidence: 99%

Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management

Banasiak¹,

Malek²

2007

Neurosurgical FOCUS

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ELSON syndrome is a rare clinical manifestation of an enlarging pituitary adenoma that can occur following bilateral adrenalectomy performed in the treatment of CD. It is characterized by skin and mucus membrane hyperpigmentation and excess ACTH secretion. Diagnostic criteria for NS have been quite variable. Some authors 77 have defined NS as high levels of ACTH associated with skin hyperpigmentation after bilateral adrenalectomy regardless of pituitary enlargement, 80 whereas others consider the presence of an expanding pituitary adenoma causing visual field disturbance as a requirement for diagnosis. 114 With modern day imaging, and for the purposes of this review, corticotroph tumor progression and high ACTH have been proposed as a basis for the diagnosis of NS. 4 Historical Perspective The first suggestion that the adrenal gland was essential for life came from Brown-Séquard, who showed in the 1850s that bilateral adrenalectomy in animals caused death within a few days. 66 Harvey Cushing 13 was the first to link adrenal hypersecretion of cortisol to the presence of a pituitary tumor. The concept of the hypothalamic-pituitary-adrenal axis evolved further with the demonstration of the existence of ACTH-releasing factor in 1955 by Saffran and Schally. 98 The first case report of the characteristic triad of NS was published by Don Nelson in 1958. 78 He described a 33-year-old woman who developed marked skin hyperpigmentation, high plasma ACTH levels, and imaging evidence of a pituitary tumor (enlarged sella on skull radiographs) 3 years after bilateral adrenalectomy for CD. In 1960, Nelson and colleagues 79 formally described the syndrome of pituitary hypersecretion of ACTH and pituitary tumor enlargement, which has since become the eponymous syndrome. Corticotropin-releasing hormone was finally isolated and sequenced by Vale and coworkers in 1981, 113 and with the availability of radioimmunoassay, the elevation of plasma ACTH levels after bilateral adrenalectomy became diagnostic for NS. Historically, patients with CD were treated with bilateral

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“…Nelson syndrome is characterized by: 1) growing residual pituitary adenoma, 2) ACTH concentration Ͼ300 mg/ dL, and 3) hyperpigmentation of the skin following bilateral adrenalectomy. 9,10 Twenty-six of 35 patients (74.3%) had a serum ACTH level less than 300 pg/mL and 9/35 patients (25.7%) had an elevated ACTH level (Table 3). Three of 35 patients (8.6%) had MRI evidence of growing residual pitu- Thirty-one of 36 patients (86.1%) were either satisfied or very satisfied with their BLA (Table 5).…”

Section: Resultsmentioning

confidence: 99%

Improved Quality of Life After Bilateral Laparoscopic Adrenalectomy for Cushing's Disease: A 10-Year Experience

Ludlam¹

2008

Yearbook of Endocrinology

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Profound amplification of secretory‐burst mass and anomalous regularity of ACTH secretory process in patients with Nelson's syndrome compared with Cushing's disease

Cited by 27 publications

References 37 publications

Leksell Gamma Knife : An Effective Non Invasive Treatment for Rare Case of Nelson’s Syndrome

Leksell Gamma Knife : An Effective Non Invasive Treatment for Rare Case of Nelson’s Syndrome

Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management

Improved Quality of Life After Bilateral Laparoscopic Adrenalectomy for Cushing's Disease: A 10-Year Experience

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