Adrenoleukodystrophy epidemiology and demographics
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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
Adrenoleukodystrophy is the most common peroxisomal disorder, with an estimated incidence of 1 in 17,000 births (male and female) and 1 in 21,000 males in the United States. The prevalence of X-linked adrenoleukodystrophy is approximately 1 in 20,000 individuals. This condition occurs with a similar frequency in all populations. The lifetime prevalence of adrenal insufficiency in ALD is ~80%.
Epidemiology and Demographics
Incidence
- Adrenoleukodystrophy is the most common peroxisomal disorder, with an estimated incidence of 1 in 17,000 births (male and female) and 1 in 21,000 males in the United States. [1]
Prevalence
- The prevalence of X-linked adrenoleukodystrophy is approximately 1 in 20,000 individuals. This condition occurs with a similar frequency in all populations.
- The lifetime prevalence of adrenal insufficiency in ALD is ~80%. [2]
Age
- Childhood cerebral type commonly occurs in 3-10 years of age.[3]
- AMN type has a median age of 28 years.[3]
- Addison disease type occurs in 0 to (greater or equal to) 10 years of age.[3]
- Cerebellar type can occur in childhood or adolescence.
Race
There is no racial predilection to adrenoleukodystrophy.
Gender
- Men are more commonly affected by adrenoleukodystrophy than women. However, women can be a carrier of the disease.
References
- ↑ Wiens K, Berry SA, Choi H, Gaviglio A, Gupta A, Hietala A; et al. (2019). "A report on state-wide implementation of newborn screening for X-linked Adrenoleukodystrophy". Am J Med Genet A. 179 (7): 1205–1213. doi:10.1002/ajmg.a.61171. PMC 6619352 Check
|pmc=value (help). PMID 31074578. - ↑ Huffnagel, Irene C; Laheji, Fiza K; Aziz-Bose, Razina; Tritos, Nicholas A; Marino, Rose; Linthorst, Gabor E; Kemp, Stephan; Engelen, Marc; Eichler, Florian (2019). "The Natural History of Adrenal Insufficiency in X-Linked Adrenoleukodystrophy: An International Collaboration". The Journal of Clinical Endocrinology & Metabolism. 104 (1): 118–126. doi:10.1210/jc.2018-01307. ISSN 0021-972X.
- ↑ 3.0 3.1 3.2 Moser HW, Raymond GV, Dubey P (2005). "Adrenoleukodystrophy: new approaches to a neurodegenerative disease". JAMA. 294 (24): 3131–4. doi:10.1001/jama.294.24.3131. PMID 16380594.