Ophanet, who are a consortium of European partners,
currently defines a condition rare when if affects 1 person per 2,000.
They list Congenital adrenal hyperplasia as a "rare disease".
More information about Congenital adrenal hyperplasia is available from Orphanet
All statistics for Congenital adrenal hyperplasia
Prevalence/Incidence of Congenital adrenal hyperplasia: Online Medical Books
16 MEDICAL BOOKS ONLINE!
Review excerpts from medical books online, free, without registration,
for more information about the prevalence and/or incidence of Congenital adrenal hyperplasia.
Adrenal hypofunction:
Causes and incidence
(Professional Guide to Diseases (Eighth Edition))
Adrenal hypofunction occurs when more than 90% of both adrenal glands are destroyed, an occurrence that typically results from an autoimmune process in which circulating antibodies react specifically against the adrenal tissue. Other causes include tuberculosis (once the chief cause; now responsible for less than 10% of adult cases), bilateral adrenalectomy, hemorrhage into the adrenal gland, neoplasms, and infections (acquired immunodeficiency syndrome, histoplasmosis, and cytomegalovirus). Rarely, a familial tendency to autoimmune disease predisposes the patient to adrenal hypofunction and other endocrinopathies.
Secondary adrenal hypofunction that results in glucocorticoid deficiency can stem from hypopituitarism (causing decreased corticotropin secretion), abrupt withdrawal of long-term corticosteroid therapy (long-term exogenous corticosteroid stimulation suppresses pituitary corticotropin secretion and results in adrenal gland atrophy), or removal of a nonendocrine, corticotropin-secreting tumor. Adrenal crisis follows when trauma, surgery, or other physiologic stress exhausts the body’s stores of glucocorticoids in a person with adrenal hypofunction.
Adrenal hypofunction affects 1 in 16,000 neonates congenitally. In adults, it affects 8 in 100,000 people, and males and females are affected equally. There’s no racial predilection.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Adrenogenital syndrome:
Causes and incidence
(Professional Guide to Diseases (Eighth Edition))
CAH is transmitted as an autosomal recessive trait that causes deficiencies in the enzymes needed for adrenocortical secretion of cortisol and, possibly, aldosterone. Compensatory secretion of corticotropin produces varying degrees of adrenal hyperplasia. In simple virilizing CAH, deficiency of the enzyme 21-hydroxylase results in underproduction of cortisol. In turn, this cortisol deficiency stimulates increased secretion of corticotropin, producing large amounts of cortisol precursors and androgens that don’t require 21-hydroxylase for synthesis.
In salt-losing CAH, 21-hydroxylase is almost completely absent. Corticotropin secretion increases, causing excessive production of cortisol precursors, including salt-wasting compounds. However, plasma cortisol and aldosterone levels — both dependent on 21-hydroxylase — fall precipitously and, in combination with the excessive production of salt-wasting compounds, precipitate acute adrenal crisis. Corticotropin hypersecretion stimulates adrenal androgens, possibly even more than in simple virilizing CAH, and produces masculinization. Other rare CAH enzyme deficiencies exist and lead to increased or decreased production of affected hormones.
CAH is the most prevalent adrenal disorder in infants and children; simple virilizing CAH and salt-losing CAH are the most common forms. Acquired adrenal virilism is rare and affects twice as many females as males. About 1 in 10,000 to 18,000 are born with CAH.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Cushing Syndrome (Adrenal Excess):
Cushing Syndrome - epidemiology
(The 5-Minute Pediatric Consult)
- Cushing disease: Female > Male
- Adrenocortical carcinoma: Female > Male
- Cushing disease: Most common cause of endogenous Cushing syndrome, accounting for 80% of Cushing syndrome in adults and children >7 years of age
- Adrenal tumor: Adrenocortical carcinomas account for >50% of Cushing syndrome in children <7 years of age. These tumors are less common in adults and children >7 years of age.
Cushing Syndrome - incidence
- 0.1–0.5/1,000,000 new pediatric cases per year
- 10 times more common in adults
>
» READ BOOK EXCERPT ONLINE »
Source: The 5-Minute Pediatric Consult, 2008
Congenital Infections:
Epidemiology
(Pediatric Infectious Disease)
CMV is the most common congenital infection, affecting 1% to 2% of all newborns.
The rate of infection in a fetus of a mother with primary infection is between
40% and 50%; the rate of infection is less than 1% if the mother has
reactivated infection. Most infected infants (90%) are asymptomatic at birth;
only 10% of these children have long-term sequelae. The most common sequela is
asymptomatic congenital CMV infection sensorineural hearing loss. This hearing
loss can be progressive and may be unilateral or bilateral.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Infectious Disease, 2004
About prevalence and incidence statistics:
The term 'prevalence' of Congenital adrenal hyperplasia usually refers to the estimated population
of people who are managing Congenital adrenal hyperplasia at any given time.
The term 'incidence' of Congenital adrenal hyperplasia refers to the annual diagnosis rate,
or the number of new cases of Congenital adrenal hyperplasia diagnosed each year.
Hence, these two statistics types can differ:
a short-lived disease like flu can have high annual incidence but low prevalence,
but a life-long disease like diabetes has a low annual incidence but high prevalence.
For more information see about prevalence and incidence statistics.
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