- Nelson's syndrome
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Nelson's syndrome Classification and external resources ICD-10 E24.1 DiseasesDB 8863 eMedicine ped/1558 Nelson's syndrome is the rapid enlargement of a pituitary adenoma that occurs after the removal of both adrenal glands.[1]
Contents
Pathophysiology
Removal of both adrenal glands, or bilateral adrenalectomy, is an operation for Cushing's syndrome. Removal of both adrenals eliminates production of cortisol, and the lack of cortisol's negative feedback can allow any preexisting pituitary adenoma to grow unchecked. Continued growth can cause mass effects due to physical compression of brain tissue, along with increased production of adrenocorticotrophic hormone (ACTH) and melanocyte stimulating hormone (MSH).
Presentation
The common signs and symptoms include muscle weakness and skin hyperpigmentation due to excess MSH. Nelson's syndrome is now rare because bilateral adrenalectomy is now only used in extreme circumstances.[2]
Treatment
Pituitary surgery is performed in some cases. The risk can also be minimized by pituitary irradiation.
History
The condition is named for the American endocrinologist Dr Don Nelson, who first reported on the phenomenon in 1960.[3]
References
- ^ Biller BM, Grossman AB, Stewart PM, et al. (July 2008). "Treatment of adrenocorticotropin-dependent Cushing's syndrome: a consensus statement". J. Clin. Endocrinol. Metab. 93 (7): 2454–62. doi:10.1210/jc.2007-2734. PMID 18413427. http://jcem.endojournals.org/cgi/content/full/93/7/2454.
- ^ Kelly PA, Samandouras G, Grossman AB, Afshar F, Besser GM, Jenkins PJ (December 2002). "Neurosurgical treatment of Nelson's syndrome". J. Clin. Endocrinol. Metab. 87 (12): 5465–9. doi:10.1210/jc.2002-020299. PMID 12466338. http://jcem.endojournals.org/cgi/pmidlookup?view=long&pmid=12466338.
- ^ Nelson D, Meakin J, Thorn G (1960). "ACTH-producing pituitary tumors following adrenalectomy for Cushing's syndrome". Ann Intern Med 52: 560–9. PMID 14426442.
Endocrine pathology: endocrine diseases (E00–E35, 240–259) Pancreas/
glucose
metabolismHypofunctiontypes: (type 1, type 2, MODY 1 2 3 4 5 6) · complications (coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy, cardiomyopathy)
insulin receptor (Rabson–Mendenhall syndrome) · Insulin resistanceHyperfunctionHypothalamic/
pituitary axesHypothalamusPituitaryanterior (Acromegaly, Hyperprolactinaemia, Pituitary ACTH hypersecretion) · posterior (SIADH) · general (Nelson's syndrome)anterior (Kallmann syndrome, Growth hormone deficiency, ACTH deficiency/Secondary adrenal insufficiency) · posterior (Neurogenic diabetes insipidus) · general (Empty sella syndrome, Pituitary apoplexy, Sheehan's syndrome, Lymphocytic hypophysitis)ThyroidHyperthyroxinemia (Thyroid hormone resistance, Familial dysalbuminemic hyperthyroxinemia) · Hashitoxicosis · Thyrotoxicosis factitia · Graves' diseaseAcute infectious · Subacute (De Quervain's, Subacute lymphocytic) · Autoimmune/chronic (Hashimoto's, Postpartum, Riedel's)ParathyroidAdrenalaldosterone: Hyperaldosteronism/Primary aldosteronism (Conn syndrome, Bartter syndrome, Glucocorticoid remediable aldosteronism) · AME · Liddle's syndrome · 17α CAHGonadsHeight Multiple Autoimmune polyendocrine syndrome (APS1, APS2) · Carcinoid syndrome · Multiple endocrine neoplasia (1, 2A, 2B) · Progeria (Werner syndrome, Acrogeria, Metageria) · Woodhouse-Sakati syndromeCategories:- Pituitary disorders
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