21-hydroxylase deficiency epidemiology and demographics
|
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency Microchapters |
|
Differentiating Congenital adrenal hyperplasia due to 21-hydroxylase deficiency from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
21-hydroxylase deficiency epidemiology and demographics On the Web |
|
American Roentgen Ray Society Images of 21-hydroxylase deficiency epidemiology and demographics |
|
FDA on 21-hydroxylase deficiency epidemiology and demographics |
|
CDC on 21-hydroxylase deficiency epidemiology and demographics |
|
21-hydroxylase deficiency epidemiology and demographics in the news |
|
Blogs on 21-hydroxylase deficiency epidemiology and demographics |
|
Directions to Hospitals Treating Congenital adrenal hyperplasia due to 21-hydroxylase deficiency |
|
Risk calculators and risk factors for 21-hydroxylase deficiency epidemiology and demographics |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Mehrian Jafarizade, M.D [2]
Overview
Epidemiology and Demographics
Incidence
- Worldwide, the incidence of 21-hydroxilase deficiency classic salt wasting type is 5 per 100,000 persons.
- Worldwide, the incidence of 21-hydroxilase deficiency classic simple or non-salt wasting type is 16.6 per 100,000 persons.
- Worldwide, the incidence of 21-hydroxilase deficiency late onset type type is 100 per 100,000 persons.[1]
Prevalence
- Prevalence varies according to ethnicity and geographic area.
- Worldwide, the prevalence of congenital adrenal hyperplasia due to 21 hydroxylase deficiency ranges from a low of 3.57 per 100,000 persons in Chinese population[2] to a high of per 100,000 persons with an average prevalence of 357 per 100,000 persons in Yupik Eskimos in Alaska.[3][1]
Race
- Congenital adrenal hyperplasia due to 21-hydroxylase deficiency usually affects individuals of the Ashkenazi Jews and Mediterranean race.
- The Ashkenazi Jews to Mediterranean race ratio is approximately 1 to 3.[4]
The classic type affects approximately 1 in 16,000 live births. NCCAH is one of the most common autosomal recessive disorders in humans and affects approximately 1 in 1000 individuals, but in up to 1–2% among inbred populations, such as Eastern European (Ashkenazi) Jews.[5]
Incidence for each region:
21-hydroxylase deficiency is more prevalent in some ethnic groups, particularly in remote geographic regions (e.g. Alaskan Yupiks). Disease incidence for each region mentioned below:[3][2][1]
- Alaska, Yupik Eskimos : 1/280
- France, La Reunion: 1/2,100
- Sweden: 1/9,800
- United States, Wisconsin: 1/11,000
- France, Lille: 1/13,000
- Japan: 1/18,000
- China: 1/28000
- United States, Texas: 1/16,000
- Scotland: 1/17,000
- Italy: 1/18,000
- New Zealand: 1/23,000
References
- ↑ 1.0 1.1 1.2 White PC, Speiser PW (2000). "Congenital adrenal hyperplasia due to 21-hydroxylase deficiency". Endocr. Rev. 21 (3): 245–91. doi:10.1210/edrv.21.3.0398. PMID 10857554.
- ↑ 2.0 2.1 Lee HH, Kuo JM, Chao HT, Lee YJ, Chang JG, Tsai CH, Chung BC (2000). "Carrier analysis and prenatal diagnosis of congenital adrenal hyperplasia caused by 21-hydroxylase deficiency in Chinese". J. Clin. Endocrinol. Metab. 85 (2): 597–600. doi:10.1210/jcem.85.2.6367. PMID 10690861.
- ↑ 3.0 3.1 Pang S, Murphey W, Levine LS, Spence DA, Leon A, LaFranchi S, Surve AS, New MI (1982). "A pilot newborn screening for congenital adrenal hyperplasia in Alaska". J. Clin. Endocrinol. Metab. 55 (3): 413–20. doi:10.1210/jcem-55-3-413. PMID 7096533.
- ↑ Pang SY, Wallace MA, Hofman L, Thuline HC, Dorche C, Lyon IC; et al. (1988). "Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency". Pediatrics. 81 (6): 866–74. PMID 3259306.
- ↑ Speiser PW, Dupont B, Rubinstein P, Piazza A, Kastelan A, New MI (1985). "High frequency of nonclassical steroid 21-hydroxylase deficiency". Am. J. Hum. Genet. 37 (4): 650–67. PMC 1684620. PMID 9556656.