Nelson's syndrome: Definition from Answers.com

Nelson's syndrome
Classification and external resources
ICD-10	E 24.1
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]

1 Pathophysiology
2 Presentation
3 Treatment
4 History
5 References

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 minimised 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 (E00-35, 240-259)

Pancreas/
glucose
metabolism

Hypofunction	Diabetes mellitus types: (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 resistance

Hyperfunction	Hypoglycemia · beta cell (Hyperinsulinism) · G cell (Zollinger-Ellison syndrome)

Hypothalamic/
pituitary axes

Hypothalamus

gonadotropin (Kallmann syndrome, Adiposogenital dystrophy) · CRH (Tertiary adrenal insufficiency) · vasopressin (Neurogenic diabetes insipidus) · general (Hypothalamic hamartoma)

Pituitary

Hyperpituitarism	anterior (Acromegaly, Hyperprolactinaemia, Pituitary ACTH hypersecretion) · posterior (SIADH) · general (Nelson's syndrome)

Hypopituitarism	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)

Thyroid

Parathyroid

Hypoparathyroidism	Pseudohypoparathyroidism

Hyperparathyroidism	Primary · Secondary · Tertiary · Osteitis fibrosa cystica

Adrenal

Hyperfunction	aldosterone: Hyperaldosteronism/Primary aldosteronism (Conn syndrome, Bartter syndrome, Glucocorticoid remediable aldosteronism) · AME · Liddle's syndrome · 17α CAH cortisol: Cushing's syndrome (Pseudo-Cushing's syndrome) sex hormones: 21α CAH · 11β CAH

Hypofunction/ Adrenal insufficiency (Addison's, WF)	aldosterone: Hypoaldosteronism (21α CAH, 11β CAH) cortisol: CAH (Lipoid, 3β, 11β, 17α, 21α) sex hormones: 17α CAH

Gonads

ovarian: Polycystic ovary syndrome · Premature ovarian failure

testicular: enzymatic (5-alpha-reductase deficiency, 17-beta-hydroxysteroid dehydrogenase deficiency) · Androgen receptor (Androgen insensitivity syndrome)

general: Hypogonadism (Delayed puberty) · Hypergonadism (Precocious puberty)

Height

Gigantism · Dwarfism/Short stature (Laron syndrome, Psychogenic dwarfism)

Multiple

Autoimmune polyendocrine syndrome (APS1, APS2) · Carcinoid syndrome · Multiple endocrine neoplasia (1, 2A, 2B) · Progeria · Woodhouse-Sakati syndrome

endocrine navs: anat/physio/dev/hormones, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

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Nelson's syndrome

Contents

Pathophysiology

Presentation

Treatment

History

References