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Topic: Congenital adrenal hyperplasia

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	eMedicine - Congenital Adrenal Hyperplasia : Article Excerpt by: Thomas A Wilson, MD
		Congenital adrenal hyperplasia caused by 11-beta-hydroxylase deficiency accounts for 5-8% of all congenital adrenal hyperplasia cases.
		The morbidity of the various forms of adrenal hyperplasia is best understood in the context of the steroidogenic pathway used by the adrenal glands and gonads (see Image 1).
		Congenital adrenal hyperplasia secondary to CYP21 deficiency is particularly common among the Yupik Eskimos.
	www.emedicine.com /ped/byname/congenital-adrenal-hyperplasia.htm (591 words)

	Congenital Adrenal Hyperplasia (CAH) - My Child Has - Children's Hospital Boston
		The overabundance of androgens is responsible for the ambiguous genitalia in females born with this condition.
		Children with CAH are treated urgently because the majority (75 percent) of these children are so-called "salt wasters." This means that the adrenal gland is not putting out enough of the mineral corticoid, which can cause the baby to rapidly lose salt.
		The overall management strategy for patients with CAH is more clear cut than treatment of other intersex conditions, because research conclusively shows that those born with internal female reproductive structures have the long-term potential to function reasonably as females from a sexual and fertility standpoint.
	www.childrenshospital.org /az/Site2190/mainpageS2190P0.html (641 words)

	Congenital Adrenal Hyperplasia (Site not responding. Last check: 2007-11-07)
		Normally, the adrenal gland converts cholesterol to cortisone and other hormones through a series of enzyme mediated reactions that are regulated through feed-back loops.
		Females with congenital adrenal hyperplasia have a normal female karyotype (46 XX).
		In the past, females affected by congenital adrenal hyperplasia often underwent surgical reconstruction to decrease the size of the clitoris and to widen the vaginal introitus.
	www.meddean.luc.edu /lumen/MedEd/urology/cah.htm (283 words)

	Congenital Adrenal Hyperplasia Information on Healthline
		Congenital adrenal hyperplasia (CAH) is a genetic disorder characterized by a deficiency in the hormones cortisol and aldosterone and an over-production of the hormone androgen.
		Congenital adrenal hyperplasia (CAH) is a form of adrenal insufficiency in which 21-hydroxylase, the enzyme that produces two important adrenal steroid hormones, cortisol and aldosterone, is deficient.
		CAH is diagnosed by a careful examination of the genitals and blood and urine tests that measure the hormones produced by the adrenal gland.
	www.healthline.com /galecontent/congenital-adrenal-hyperplasia (822 words)

	Congenital Adrenal Hyperplasia (Site not responding. Last check: 2007-11-07)
		Congenital adrenal hyperplasia (CAH) is a family of autosomal recessive disorders of adrenal steroidogenesis.
		The frequency of unexplained deaths of male infants in families later recognized to be at risk for CAH demonstrates the risk of death from adrenal crisis in newborns with CAH.
		The increase in adrenal androgens affects the HPG axis in two ways: in the short term it suppresses the axis through the negative feed-back effects of sex steroids; in the long term it activates the axis by advancing somatic and skeletal maturation with resultant secondary central precocious puberty.
	home.coqui.net /myrna/cah.htm (2144 words)

	Congenital adrenal hyperplasia (CAH)
		Congenital adrenal hyperplasia (CAH) refers to any of several autosomal recessive diseases resulting from defects in steps of the synthesis of cortisol from cholesterol by the adrenal glands.
		Congenital adrenal hyperplasia (CAH) is a form of adrenal insufficiency in which the enzyme that produces two important adrenal steroid hormones, cortisol and aldosterone, is deficient.
		CAH, a genetic disorder, is the most common adrenal gland disorder in infants and children, occurring in one in 10,000 total births worldwide.
	endocrine-disorders.health-cares.net /congenital-adrenal-hyperplasia.php (639 words)

	Congenital Adrenal Hyperplasia: Endocrine and Metabolic Disorders in Children: Merck Manual Professional
		Congenital adrenal hyperplasia is a group of genetic disorders, each characterized by inadequate synthesis of cortisol, aldosterone, or both.
		The salt-wasting form (sometimes called the classic form of congenital adrenal hyperplasia) is the most severe form of 21-hydroxylase deficiency; aldosterone is not secreted and salt is lost, leading to hyponatremia, hyperkalemia, and increased plasma renin activity.
		For adrenal crisis in infants, urgent therapy with IV fluids is needed.
	www.merck.com /mmpe/sec19/ch282/ch282e.html (1371 words)

	Congenital Adrenal Hyperplasia \| Caremark Health Resources
		CAH is a genetic disorder characterized by a deficiency in the hormones cortisol and aldosterone and an over-production of the hormone androgen, which is present at birth and affects sexual development.
		Congenital adrenal hyperplasia (CAH) is a form of adrenal insufficiency in which the enzyme that produces two important adrenal steroid hormones, cortisol and aldosterone, is deficient.
		CAH, a genetic disorder, is the most common adrenal gland disorder in infants and children, occurring in one in 10,000 total births worldwide.
	healthresources.caremark.com /topic/adrenalhyper (1201 words)

	Congenital adrenal hyperplasia Medical Information
		Congenital adrenal hyperplasia refers to a group of inherited adrenal gland disorders.
		Some forms of congenital adrenal hyperplasia are more severe and cause adrenal crisis in the newborn due to salt wasting.
		Parents of children with congenital adrenal hyperplasia should be aware of the side effects of steroid therapy.
	www.drugs.com /enc/congenital_adrenal_hyperplasia.html (919 words)

	Congenital adrenal hyperplasia - Better Health Channel.
		Congenital adrenal hyperplasia (CAH) is an inherited group of disorders that involves any one of the enzymes necessary to manufacture cortisol by the adrenal glands.
		Female infants born with either salt-losing or non-salt-losing CAH commonly have ambiguous (not clearly female or male) genitals due to an excess of the male sex hormone androgen.
		Congenital adrenal hyperplasia (CAH) is an inherited group of disorders characterised by incorrect amounts of adrenal gland hormones.
	www.betterhealth.vic.gov.au /bhcv2/bhcarticles.nsf/pages/Congenital_adrenal_hyperplasia?Open (1309 words)

	Congenital adrenal hyperplasia (CAH)
		Congenital adrenal hyperplasia (CAH) is a relatively common disease that can affect normal growth and development, and may influence how your body deals with the stress of illness or injury.
		CAH is a group of inherited genetic disorders that cause a block in the body's ability to make enough of the "stress" hormone, cortisol.
		CAH occurs in two forms: severe or "classical" and mild or "non-classical." The effects of classical CAH occur as a result of the hormonal imbalance of cortisol, aldosterone and androgens.
	www.hormone.org /public/cah.cfm (843 words)

	Congenital Adrenal Hyperplasia: Not Really a Zebra American Family Physician - Find Articles
		Mild congenital adrenal hyperplasia, however, is common, affecting one in 100 to 1,000 persons in the United States and frequently eluding diagnosis.
		Congenital adrenal hyperplasia is diagnosed by demonstration of excess cortisol precursors in the serum during an adrenal corticotropic hormone challenge.
		Classical congenital adrenal hyperplasia is rare, affecting only one in 14,000 patients, but mild forms of the disease may occur in one of every 100 to 1,000 persons.1,2 The condition is caused by a deficient synthesis of cortisol; most cases are related to 21-hydroxylase or 11-b hydroxylase deficiency3-5 (Figure 1).
	www.findarticles.com /p/articles/mi_m3225/is_5_59/ai_54952074 (740 words)

	CONGENITAL ADRENAL HYPERPLASIA PEDIATRIC ONCALL
		In view of suspicion of congenital adrenal hyperplasia of 21 hydroxylase deficiency — a serum 17 hydroxy progesterone was done which was elevated [120 ng/ml (Normal = 0.7-0.77 ng/ml)] confirming the diagnosis.
		Thus, he was suspected as Congenital adrenal hyperplasia → non classic variety in a male child and treated with T. Fludrocortisone and Hydrocortisone.
		Congenital adrenal hyperplasia is an autosomal recessive disorder of adrenal corticosteroid biosynthesis due to deficiency of a particular enzyme.
	www.pediatriconcall.com /fordoctor/casereports/CONGENITAL_ADRENAL_HYPERPLASIA.asp (949 words)

	Congenital Adrenal Hyperplasia
		Each adrenal gland is made up of two regions, the inner region (called the medulla) which produces adrenaline and the outer region (called the cortex) which produces adrenal steroid hormones.
		If a person with CAH is sick with a fever of 101°F or higher, or has a serious injury (such as a broken bone), he or she may need additional glucocorticoid treatment.
		Topics of particular concern to some CAH patients are gaining an understanding of their medical condition, and the life-long requirement of medication for normal growth, pubertal development, fertility (for women), genital surgery (for women) and sexuality.
	www.hopkinschildrens.org /specialties/categorypages/cah/printable.html (2854 words)

	Congenital Adrenal Hyperplasia (CAH), CARES Foundation
		Congenital Adrenal Hyperplasia is a family of inherited disorders affecting the adrenal gland.
		The remaining 25% of those with classical CAH who produce sufficient aldosterone are called “simple virilizers.”; The nonclassical form of CAH is not life threatening, but can affect puberty and growth in children and can cause infertility in males and females as well as other symptoms affecting quality of life.
		Other forms of enzyme deficiency that belong to the congenital adrenal hyperplasia family of disorders, such as 3-beta hydroxylase deficiency, 11-beta hydroxylase deficiency and 17 alpha-hydroxylase deficiency, are much less common and are not discussed here.
	www.caresfoundation.org /what.html (748 words)

	Congenital Adrenal Hyperplasia (CAH)
		Congenital Adrenal Hyperplasia (CAH) is a family of autosomal recessive disorders characterized by the inability to synthesize cortisol, and in most instances, also the inability to synthesize the salt-retaining hormone, aldosterone.
		The most common form of CAH (more than 90% of cases) is caused by a deficiency of the adrenal enzyme 21-hydroxylase (21-OH) and is identified by assessing the level of 17-alpha-hydroxyprogesterone (17-OHP) in blood.
		CAH is the most common cause of ambiguous genitalia in females, and can cause acute life-threatening adrenal crisis in both males and females in the neonatal period.
	www.dhss.mo.gov /Lab/Newborn/CAH.html (615 words)

	Congenital adrenal hyperplasia :: Web Articles :: (Site not responding. Last check: 2007-11-07)
		Congenital adrenal hyperplasia (CAH) refers to any of several autosomal recessive diseases resulting from defects in steps of the synthesis of cortisol from cholesterol by the adrenal glands.
		CAH due to deficiencies of other enzymes than 21-hydroxylase present many of the same management challenges as 21-hydroxylase deficiency, but some involve mineralocorticoid excess or sex steroid deficiency.
		Congenital adrenal hyperplasia, which also dates to the first half of the century, has become the preferred term to reduce ambiguity and to emphasize the underlying pathophysiology of the disorders.
	www.webarticles.com /Health/Women/Congenital-adrenal-hyperplasia (1666 words)

	Congenital Adrenal Hyperplasia
		Congenital adrenal hyperplasias (CAH) are a group of heritable disorders associated with an inability or deficiency in the ability to produce cortisol.
		In the most common cases, adrenal hyperplasia also involves a deficiency in aldosterone, which results in mild to severe loss of body sodium.
		CAH due to 21-hydroxylase deficiency is seen in roughly 1 of every 15,000 live births worldwide; it is a relatively common disorder in humans.
	arbl.cvmbs.colostate.edu /hbooks/pathphys/endocrine/adrenal/cah.html (939 words)

	Congenital adrenal hyperplasia - WrongDiagnosis.com
		Congenital genetic disease with insufficiency of the adrenal glands
		Congenital adrenal hyperplasia (CAH) is a genetic disease that affects the adrenal glands.
		Congenital adrenal hyperplasia is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH).
	www.wrongdiagnosis.com /c/congenital_adrenal_hyperplasia/intro.htm (797 words)

	Congenital Adrenal Hyperplasia
		Congenital adrenal hyperplasia is a genetic disorder of the adrenal glands.
		Congenital adrenal hyperplasia is caused by an error on a single gene.
		Congenital adrenal hyperplasia is the most common autosomal recessive genetic disorder in humans.
	www.nichd.nih.gov /health/topics/congenital_adrenal_hyperplasia.cfm (343 words)

	NEW DEVELOPMENTS IN THE TREATMENT AND DIAGNOSIS OF CONGENITAL ADRENAL HYPERPLASIA (Site not responding. Last check: 2007-11-07)
		Congenital adrenal hyperplasia (CAH), also termed adrenogenital syndrome in older literature, is a common inherited form of adrenal insufficiency.
		Since the incidence of classic CAH worldwide is about 1 in 5,000 male births (or 1 in 10,000 total births), this amounts to a substantial number of potentially preventable infant deaths.
		Since CAH is a recessive disease, one has a 50% chance of inheriting a mutant gene from each carrier parent, and the risk of an affected child is 25% in each pregnancy.
	www.medhelp.org /www/nadf5.htm (2258 words)

	Congenital Adrenal Hyperplasia - Symptoms, Treatment and Prevention
		The term congenital adrenal hyperplasia (CAH) refers to a complex series of rare but well-studied enzymatic errors of metabolism with deficient levels of different enzymes involved in the synthesis of cortisol (hydrocortisone).
		In several forms of CAH in which adrenomegaly produces abnormally large amounts of androgen (a hormone that stimulates the activity of male sex organs), abnormalities of sexual development may be the most conspicuous consequence, particularly masculinization of the external genitalia in females.
		In congenital lipoid hyperplasia (adrenal cortex with male pseudo-hermaphroditism [false presence of both ovarian and testicular tissue]), the most prominent feature is male pseudohermaphroditism.
	www.healthscout.com /ency/428/670/main.html (678 words)

	Congenital Adrenal Hyperplasia
		Congenital adrenal hyperplasia was once considered a rare inherited disorder with severe manifestations.
		Mild congenital adrenal hyperplasia, however, is common, affecting up to 1% of all women in the persons in the United States and frequently eluding diagnosis.
		Congenital adrenal hyperplasia is diagnosed by demonstration of excess cortisol precursors in the blood, especially after a test injection of the drug ACTH.
	www.aocd.org /skin/dermatologic_diseases/congenital_adrenal.html (318 words)

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