Adrenoleukodystrophy natural history, complications and prognosis: Difference between revisions
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==Natural History== | ==Natural History== | ||
*Adrenoleukodystrophy is a progressive disease as the risk of symptoms increases with age but its manifestations are highly variable. | |||
*Most of the male patients presents with adrenal insufficiency (Addison disease) in childhood (80% prevalence) while those in adulthood presents with signs of myelopathy. Women with the ALD usually presents with myelopathy, other manifestations such as adrenal insufficiency are unusual. <ref name="KempHuffnagel2016">{{cite journal|last1=Kemp|first1=Stephan|last2=Huffnagel|first2=Irene C.|last3=Linthorst|first3=Gabor E.|last4=Wanders|first4=Ronald J.|last5=Engelen|first5=Marc|title=Adrenoleukodystrophy – neuroendocrine pathogenesis and redefinition of natural history|journal=Nature Reviews Endocrinology|volume=12|issue=10|year=2016|pages=606–615|issn=1759-5029|doi=10.1038/nrendo.2016.90}}</ref> | |||
==Complications== | ==Complications== | ||
Revision as of 15:33, 18 June 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
Natural History
- Adrenoleukodystrophy is a progressive disease as the risk of symptoms increases with age but its manifestations are highly variable.
- Most of the male patients presents with adrenal insufficiency (Addison disease) in childhood (80% prevalence) while those in adulthood presents with signs of myelopathy. Women with the ALD usually presents with myelopathy, other manifestations such as adrenal insufficiency are unusual. [1]
Complications
- Adrenal crisis
- Vegetative state (long-term coma)
Prognosis
The childhood form of X-linked adrenoleukodystrophy is a progressive disease that leads to a long-term coma (vegetative state) about 2 years after neurological symptoms develop. The child can live in this condition for as long as 10 years until death occurs.
The other forms of this disease are milder.
References
- ↑ Kemp, Stephan; Huffnagel, Irene C.; Linthorst, Gabor E.; Wanders, Ronald J.; Engelen, Marc (2016). "Adrenoleukodystrophy – neuroendocrine pathogenesis and redefinition of natural history". Nature Reviews Endocrinology. 12 (10): 606–615. doi:10.1038/nrendo.2016.90. ISSN 1759-5029.