Abnormal SEX DIFFERENTIATION or congenital DISORDERS OF SEX DEVELOPMENT caused by abnormal levels of steroid hormones expressed by the GONADS or the ADRENAL GLANDS, such as in CONGENITAL ADRENAL HYPERPLASIA and ADRENAL CORTEX NEOPLASMS. Due to abnormal steroid biosynthesis, clinical features include VIRILISM in females; FEMINIZATION in males; or precocious sexual development in children.
A characteristic symptom complex.

KORSAKOFF'S SYNDROME ASSOCIATED WITH ADRENAL VIRILISM. (1/3)

Korsakoff's syndrome of obscure etiology was observed in a 34-year-old single woman with an 11-year history of hirsutism and mood swings, and previous hospitalizations for mania three years ago and depression 11 years ago.Recently the virilism had intensified with increased muscularity and coarsening of facial features. The 24-hour urinary 17-ketosteroids ranged between 14.4 mg. and 21.5 mg. and were suppressed by dexamethasone. The 17-hydroxycorticosteroid excretion was normal. These and other findings suggested a diagnosis of adrenal virilism due to adrenocortical hyperplasia. In the absence of other discernible causes it appeared that the adrenal pathology was responsible for the Korsakoff's syndrome. Both conditions responded well to glucocorticoid therapy although low doses were necessary to avoid mania.It is speculated that the encephalopathy was due to an associated adrenal insufficiency. Although hypoadrenalism is accepted as a complication of only the infant form of adrenal virilism, it is noteworthy that this patient had pathological pigmentation of her skin.  (+info)

Microscope-assisted reduction clitoroplasty used to treat two patients with clitoromegaly. (2/3)

We report 2 cases of clitoromegaly, 1 in a patient with true hermaphroditism, and the other in a patient with adrenogenital syndrome. Both were treated surgically with reduction clitoroplasty. There are 3 different clitoroplasty procedures: clitorectomy, clitoral recession, and reduction clitoroplasty. Reduction clitoroplasty with preservation of the neurovascular bundle is considered superior in terms of formation of the external genitals and sensation. However, the disadvantages are that detachment of the neurovascular bundle from the clitoral shaft is difficult and that there is a high possibility of sensory and blood flow disorders in the clitoris. In an attempt to achieve safe and reliable surgical manipulation, we used a surgical microscope (OPMI 6-SDFC, Carl Zeiss Surgical GmbH, magnification x8) to detach the neurovascular bundle from the clitoral shaft in our 2 patients. Our impression is that our efforts were extremely effective. Furthermore, our experience leads us to believe that the procedure for neurovascular bundle detachment required in reduction clitoroplasty is not particularly difficult if performed with a surgical microscope by a plastic surgeon who regularly performs microsurgery. Because the procedure can be performed simply and safely, we believe that reduction clitoroplasty with preservation of the neurovascular bundle is the best overall of the 3 clitoroplasty procedures.  (+info)

Role of a disordered steroid metabolome in the elucidation of sterol and steroid biosynthesis. (3/3)

 (+info)

Adrenogenital syndrome (AGS) is a group of inherited disorders characterized by excessive production of androgens (male sex hormones) by the adrenal glands. The adrenal glands are located on top of the kidneys and are responsible for producing several essential hormones that regulate various bodily functions, including cortisol, aldosterone, and androgens.

In AGS, there is a defect in one of the enzymes involved in the production of these hormones, leading to an accumulation of precursor steroids and overproduction of androgens. This excess androgen can cause a variety of symptoms, including early puberty, rapid growth, and virilization (the development of male secondary sexual characteristics) in both males and females.

There are several different types of AGS, which are classified based on the specific enzyme deficiency involved. The most common form is congenital adrenal hyperplasia (CAH), which results from a deficiency in the enzyme 21-hydroxylase. Other forms of AGS include 11-beta-hydroxylase deficiency, 3-beta-hydroxysteroid dehydrogenase deficiency, and 17-alpha-hydroxylase deficiency.

Treatment for AGS typically involves replacing the missing hormones with medications such as corticosteroids and mineralocorticoids, as well as managing any symptoms that arise from excess androgen production. In some cases, surgery may be necessary to correct genital abnormalities or other physical manifestations of the disorder.

A syndrome, in medical terms, is a set of symptoms that collectively indicate or characterize a disease, disorder, or underlying pathological process. It's essentially a collection of signs and/or symptoms that frequently occur together and can suggest a particular cause or condition, even though the exact physiological mechanisms might not be fully understood.

For example, Down syndrome is characterized by specific physical features, cognitive delays, and other developmental issues resulting from an extra copy of chromosome 21. Similarly, metabolic syndromes like diabetes mellitus type 2 involve a group of risk factors such as obesity, high blood pressure, high blood sugar, and abnormal cholesterol or triglyceride levels that collectively increase the risk of heart disease, stroke, and diabetes.

It's important to note that a syndrome is not a specific diagnosis; rather, it's a pattern of symptoms that can help guide further diagnostic evaluation and management.

Elevated urine pregnanetriol levels suggest adrenogenital syndrome. In monitoring treatment with cortisol replacement, elevated ...
The condition was known at the time as "adreno-genital syndrome", although the term now used is congenital adrenal hyperplasia ... "The adreno-genital syndrome associated with cortical hyperplasia; the results of unilateral adrenalectomy". British Journal of ...
"Neonatal cortical insufficiency (Addison's disease) associated with the adrenogenital syndrome." The Journal of Pediatrics 18.6 ... Infertility Micropenis Orchitis Paraphimosis Penile fracture Peyronie's disease Phimosis Post-vasectomy pain syndrome Priapism ...
Uses include conditions such as adrenocortical insufficiency, adrenogenital syndrome, high blood calcium, thyroiditis, ...
Medicine portal Transgender portal Transgender voice therapy List of transgender-related topics Adrenogenital syndrome Speech ...
Genital findings in the female pseudo-hermaphroditism of the congenital adrenogenital syndrome; morphology, frequency, ... It can also be caused by the autosomal recessive congenital disorder known as Fraser syndrome. In acquired clitoromegaly, the ... van Haelst MM, Scambler PJ, Hennekam RC (December 2007). "Fraser syndrome: a clinical study of 59 cases and evaluation of ... "Polycystic Ovarian Syndrome". Retrieved 2008-09-28. Horejsí J (June 1997). "Acquired clitoral enlargement. Diagnosis and ...
... , sold under the brand name Florinef, among others, is a corticosteroid used to treat adrenogenital syndrome, ... Fludrocortisone has been used in the treatment of cerebral salt-wasting syndrome. It is used primarily to replace the missing ... Fludrocortisone is also a confirmation test for diagnosing Conn's syndrome (aldosterone-producing adrenal adenoma), the ... fludrocortisone is the first-line of treatment for orthostatic intolerance and postural orthostatic tachycardia syndrome (POTS ...
Adrenal insufficiency Adrenal macropolyadenomatosis Adrenal medulla neoplasm Adrenocortical carcinoma Adrenogenital syndrome ... Pande syndrome Aarskog syndrome Aase-Smith syndrome Aase syndrome Abasia ABCD syndrome Abdallat-Davis-Farrage syndrome ... syndrome Akesson syndrome Aksu-Stckhausen syndrome Al Awadi Teebi Farag syndrome Al Frayh Facharzt Haque syndrome Al Gazali Al ... Alien hand syndrome Alkaptonuria Allain-Babin-Demarquez syndrome Allan-Herndon-Dudley syndrome Allanson-Pantzar-McLeod syndrome ...
... may refer to: Kocher-Debré-Semelaigne syndrome Adrenogenital syndrome Myostatin mutation resulting in ... hypertrophy and increased strength This disambiguation page lists articles associated with the title Infant hercules syndrome. ...
... to detect adrenogenital syndrome, also known as congenital adrenal hyperplasia Galactosemia The test uses the growth of a ...
... a horse disease Adrenogenital syndrome Aicardi-Goutières syndrome, a rare genetic disorder AGS-17, the Soviet-designed ...
... adrenogenital syndrome MeSH C19.391.775.260 - freemartinism MeSH C19.391.775.309 - gonadal dysgenesis MeSH C19.391.775.309.193 ... Kallmann syndrome MeSH C19.391.482.629 - Klinefelter syndrome MeSH C19.391.482.814 - sexual infantilism MeSH C19.391.630.050 - ... digeorge syndrome MeSH C19.700.159.750 - diabetes insipidus, neurogenic MeSH C19.700.159.875 - wolfram syndrome MeSH C19.700. ... cushing syndrome MeSH C19.053.800.604 - hyperaldosteronism MeSH C19.053.800.604.249 - bartter syndrome MeSH C19.246.099.500 - ...
Пішак, В.П. (orcid.org/0000-0002-0637-6936) and Ризничук, М.О. (orcid.org/0000-0002-3632-2138) (2017) Adrenogenital syndrome: ... adrenogenital syndrome; diagnosis; adrenal cortex; virilization. Subjects:. Science and knowledge. Organization. Computer ... adrenogenital syndrome (AGS). Deficiency of any of these enzymes or transport proteins leads to partial or complete loss of ...
Testicular tumor of the adrenogenital syndrome (TTAGS) has also been referred to as testicular adrenal rest tumors (TARTs). ...
Elevated urine pregnanetriol levels suggest adrenogenital syndrome. In monitoring treatment with cortisol replacement, elevated ...
Disorders of sex development and adrenogenital syndrome clinic. Universitätsmedizin Mannheim. Więcej informacji ...
Congenital adrenogenital syndrome (ie, CAH). * Enzymatic defects in testosterone synthesis (ie, 5-alpha reductase deficiency) ...
Adrenogenital Syndrome * Amenorrhea * Anovulation * Anovulatory Bleeding * Assisted Reproductive Technology (ART) * ...
Adrenogenital Syndrome * Androgen Excess * Androgen Insensitivity * Bartter Syndrome * Calcium Metabolism * Carcinoid Syndrome ...
Adrenogenital syndrome or adrenocortical hyperplasia. *Cushing syndrome. *Pheochromocytoma. Pancreas:. *Diabetes mellitus. * ...
Congenital abnormality of steroid metabolism (eg, adrenogenital syndrome, 17?-hydroxylase defect). Increased flow of distal ...
Virilization (Adrenogenital Syndrome) (Merck & Co., Inc.) Adrenal Gland Disorders/Specifics ... Adrenal Gland Disorders ... ...
Adrenogenital syndrome or adrenocortical hyperplasia. *Cushing syndrome. *Pheochromocytoma. Pancreas:. *Diabetes mellitus. * ...
Congenital adrenal hyperplasia, also known as adrenogenital syndrome, is a common cause of female pseudohermaphroditism. This ... reproductive system disease congenital adrenal hyperplasia androgen insensitivity syndrome intersex vanishing testes syndrome ... In this syndrome, because of a lack of testosterone directing fetal development toward a normal male configuration, genetic ... In some persons, tissue receptors for androgens are absent or reduced, forming a spectrum of syndromes of partial to complete ...
Adrenogenital syndrome. 1. Alcoholism. 2. Alcohol use. 2. Anaemia. 9. Anaemia of pregnancy. 3. ...
Congenital Adrenal Hyperplasia (Adrenogenital Syndrome) (1) * Congestive Heart Failure (Heart Failure) (1) ... Multiple System Atrophy (MSA or Shy-Drager Syndrome or Multi-System Degeneration) (1) ...
... losing adrenogenital syndrome. It does not have marketing authorisation in the UK for treating orthostatic hypotension; ... Orthostatic hypotension may be idiopathic (Bradbury-Eggleston syndrome) or may arise as a result of disorders affecting the ... A quarter of participants had multiple system atrophy (formerly Shy-Drager syndrome), 23% had pure autonomic failure (Bradbury- ... The most common diagnosis was diabetes (27.8%), followed by Parkinsons disease (22.7%), Bradbury-Eggleston syndrome (20.6%), ...
myVMC, the online resource providing you with the latest and most comprehensive information on the field of Endocrinology.
... syndrome 255.13 Bartters syndrome 255.14 Other secondary aldosteronism 255.2 Adrenogenital disorders Adrenogenital syndromes, ... DiGeorges syndrome Pharyngeal pouch syndrome Thymic hypoplasia 279.12 Wiskott-Aldrich syndrome 279.13 Nezelofs syndrome ... 277.5 Mucopolysaccharidosis Gargoylism Hunters syndrome Hurlers syndrome Lipochondrodystrophy Maroteaux-Lamy syndrome Morquio ... syndrome Scheies syndrome 277.6 Other deficiencies of circulating enzymes Hereditary angioedema 277.7 Dysmetabolic syndrome X ...
Adducted Thumbs Syndrome + adenine phosphoribosyltransferase deficiency Adie syndrome Adrenogenital Syndrome + adult ... urofacial syndrome + A syndrome that is characterized by inverted facial expressions in association with a severe and early- ... Cerebellar Vermis Aplasia with Associated Features suggesting Smith-Lemli-Opitz Syndrome and Meckel Syndrome ... urofacial syndrome (DOID:0050816). Annotations: Rat: (2) Mouse: (2) Human: (2) Chinchilla: (2) Bonobo: (2) Dog: (2) Squirrel: ( ...
Adrenogenital Syndrome). By Ashley B. Grossman , MD, University of Oxford; Fellow, Green-Templeton College ... Cushing syndrome Cushing Syndrome Cushing syndrome is a constellation of clinical abnormalities caused by chronic high blood ... Polycystic ovary syndrome is a clinical syndrome typically characterized by anovulation or oligo-ovulation, signs of androgen ... Cushing disease is Cushing syndrome that results from... read more results, with suppression of adrenocorticotropic hormone ( ...
O Adrenogenital syndrome,O Adult onset,O Adult onset sensorineural hearing impairment,O Adult-onset night blindness,O Advanced ... O Transient myeloproliferative syndrome,O Transient neonatal diabetes mellitus,O Transient nephrotic syndrome,O Transient ... O Budd-Chiari syndrome,O Bulbar palsy,O Bulbar signs,O Bulbar urethral stricture,O Bulbous nose,O Bulbous tips of toes,O ... O Sick sinus syndrome,O Sickled erythrocytes,O Sideroblastic anemia,O Signet ring sign,O Silver-gray hair,O Simple febrile ...
Adrenogenital Syndrome. 8 (.25%). Aids Encephalopathy. 8 (.25%). Amnesia. 8 (.25%). Anaemia Macrocytic. 8 (.25%) ...
... and testicular tumors of adrenogenital syndrome (bilateral, multifocal, clinical symptoms, laboratory findings, shrink after ... syndromes, grading and molecular patterns. It then discusses all known fibroblastic / myofibroblastic, fibrohistiocytic and ...
In 1950, his successful treatment of adrenogenital syndrome with small doses of cortisone brought him widespread recognition. ...
... and adrenogenital syndromes.. Classification. Class I. ...
Adrenogenital syndrome. , Circumplex Model of Marital and Family Systems. item.page.sponsorship. "This research was supported ...
Glucocorticoids: Cortisol associated w/ Cushing syndrome. Adrenocortical Androgens associated w/ Virilizing syndromes. ... Adrenogenital Syndromes:. *Causes for Excess Androgens:. *Primary gonadal disorders. *Primary adrenal disorder: Neoplasm, ... Type I polyglandular syndrome  mutations of autoimmune regulator (AIRE) gene. *Type II polyglandular syndrome  Associated w/ ... 10% arise in association w/ familial syndromes e.g. MEN 2A and 2B syndromes ...
Bongiovanni, Alfred M.; Root, Allen W. (1963). "The Adrenogenital Syndrome". New England Journal of Medicine 268 (23): 1283.doi ... HI is usually isolated but may be rarely part of a genetic syndrome (e.g. Beckwith-Wiedemann syndrome, Sotos syndrome etc.). ... However, heart defects can be part of genetic and chromosome syndromes. Some of these syndromes may be passed down through ... Klinefelters syndrome, Sex assignment, syndrome, XY, XY sex-determination system on February 25, 2013, Leave a Comment » ...
  • Cushing Syndrome Cushing syndrome is a constellation of clinical abnormalities caused by chronic high blood levels of cortisol or related corticosteroids. (msdmanuals.com)
  • Virilization (Adrenogenital Syndrome) (Merck & Co., Inc. (nih.gov)
  • Adrenal virilism is a syndrome in which excessive adrenal androgens cause virilization. (msdmanuals.com)
  • Testicular tumor of the adrenogenital syndrome (TTAGS) has also been referred to as testicular adrenal rest tumors (TARTs). (clinicalpub.com)
  • Cushing disease is Cushing syndrome that results from. (msdmanuals.com)
  • Florinef is used for treating adrenocortical insufficiency in Addison disease and for treating salt-losing adrenogenital syndrome. (canadiandrugcenter.com)
  • It starts with approaches to diagnosis, syndromes, grading and molecular patterns. (pathologyoutlines.com)
  • Cushing's syndrome, and Hyperglycemia: Monitor patients for these conditions with chronic use. (recallguide.org)
  • Congenital abnormality of steroid metabolism (eg, adrenogenital syndrome, 17? (unboundmedicine.com)
  • orcid.org/0000-0002-3632-2138 ) (2017) Adrenogenital syndrome: molecular mechanisms of development Міжнародний ендокринологічний журнал, 2 (13). (iitta.gov.ua)
  • Adrenogenital Syndrome.Nonendocrine Diseases: Rheumatoid Arthirits. (drugbook.in)
  • Pulmonary Edema, Accelerate Lung Maturation And Surfactant Production In The Foetus And Prevent Respiratory Distress Syndrome At Birth.Infective Diseases E.g. (drugbook.in)
  • Pemphigus Vulgaris, Exfoliative Dermatitis, Stevens Johnson Syndrome.Intestinal Diseases E.g. (drugbook.in)
  • Increased testosterone levels can lead to polycystic ovarian syndrome (PCOS). (familyapp.com)
  • PCOS has not yet been fully researched and there are different types of the syndrome. (familyapp.com)
  • In 1950, his successful treatment of adrenogenital syndrome with small doses of cortisone brought him widespread recognition. (jhmi.edu)
  • Congenital adrenal hyperplasia , also known as adrenogenital syndrome, is a common cause of female pseudohermaphroditism. (britannica.com)
  • Elevated urine pregnanetriol levels suggest adrenogenital syndrome. (wikipedia.org)
  • The Androgen Insensitivity Syndrome Support Group is an English support group for women with androgen insensitivity syndrome and related intersex variations. (thisisintersex.org)
  • Adrenogenital syndrome (AHS) develops with congenital adrenal hyperplasia caused by hyperproduction of ACTH. (synthroidnews.net)
  • Fludrocortisone acetate tablets, 0.1 mg are indicated as partial replacement therapy for primary and secondary adrenocortical insufficiency in Addison's disease and for the treatment of salt-losing adrenogenital syndrome. (nih.gov)
  • Florinef is used for treating adrenocortical insufficiency in Addison disease and for treating salt-losing adrenogenital syndrome. (globalrxstore.com)
  • Adrenogenital syndrome is a recessive genetic autosomal disorder in which enzyme production is disrupted causing growth in the adrenal glands and ambiguous genitalia. (alleydog.com)
  • Adrenogenital syndrome (AGS): life-threatening, hereditary disorder affecting hormone production by the adrenal glands. (pns.nl)
  • Breaking down the name of this disorder, "acro" means "peak" in Greek and refers to the "peaked" hands of some people with this syndrome. (the-medical-dictionary.com)
  • Cushing's syndrome is a hormonal disorder. (icd9data.com)
  • In both sexes, overall rates of other disorders of adrenal gland and Cushing's syndrome were lower than for adrenal insufficiency but higher than for hyperaldosteronism, adrenogenital disorders, and adrenomedullary hyperfunction. (health.mil)
  • 1 for adrenal insufficiency to 5.5 for androgenital disorders and Cushing's syndrome. (health.mil)
  • Among females, rates of Cushing's syndrome and other disorders of adrenal gland were higher among non-Hispanic white service members compared with those in other race/ethnicity groups. (health.mil)
  • Adrenogenital effects of adrenocortical hormones can result in bodily disorders. (wordinfo.info)
  • The adrenogenital syndrome characterizes a group of ailments whereby an unusual production of steroid hormones by the adrenal cortex leads to a deviation of sexual characteristics, particularly those of virilization of females. (wordinfo.info)
  • Women with polycystic ovary syndrome tend to have elevated levels of DHEAS. (wikidoc.org)
  • With cushing's syndrome, there's too much cortisol, while with addison's disease, there is too little. (icd9data.com)
  • 14. The testicular 'tumour' of adrenogenital syndrome--a persistent diagnostic pitfall. (nih.gov)
  • Diagnosis: testicular tumor of adrenogenital syndrome due to 11-beta-hydroxylase deficiency. (nih.gov)
  • 20. [Unilateral testicular tumor in adrenogenital syndrome]. (nih.gov)
  • Kona syndrome (primary aldosteronism) develops in 1/3 cases of hyperfunction of the adrenal cortex. (synthroidnews.net)
  • Genetic syndromes causing hypertrichosis (consider especially in children). (cyberderm.net)
  • Test to distinguish Cushing's syndrome due to pituitary ACTH excess from Cushing's syndrome due to other causes. (drugs.com)
  • Endogenous cushing syndrome or spontaneous hypercortisolism is divided into two groups, those due to an excess of adrenocorticotropin and those that are acth-independent. (icd9data.com)
  • Psychosocial problems, tendency towards folliculitis after shaving, diabetes and cancer in polycystic ovarian syndrome. (cyberderm.net)
  • Claims have been found to link low levels of DHEA to obesity, metabolic syndrome, abnormal cholesterol levels and increased waistline. (wikidoc.org)
  • A syndrome caused by high levels of cortisol in the blood either due to excessive production and secretion of corticosteroids secondary to pituitary or adrenocortical neoplasms, or intake of glucocorticoid drugs. (icd9data.com)
  • Acute adrenal insufficiency - adrenaline crisis (Waterhausen-Frideriksen syndrome) - a rapidly progressive state, clinically occurring in the form of shock. (synthroidnews.net)
  • Yes, given her age and who present in half of females with trichomoniasis develop a syndrome characterized essentially as a resultant pneumothorax in the other hand, the term hypervolemia generally denotes increased extra-cellular volume is inversely proportional to the previously mentioned clinical findings. (albionfoundation.org)
  • Sexual precocity, fibrous dysplasia, and patchy pigmentation occur in McCune-Albright syndrome where hyperthyroidism is common. (mhmedical.com)
  • PMID- 5428655 TI - Serious ophthalmological complications in the Ehlers-Danlos syndrome. (nih.gov)
  • Sodium bicarbonate for metabolic errors, adrenogenital syndrome disseminated intravascular coagulation. (albionfoundation.org)
  • For example, Snyder Robinson Syndrome is a new disease SCR that is mapped to the MeSH descriptor Mental Retardation, X-Linked. (bvsalud.org)
  • Apert Syndrome , virtually synonymous with Acrocephalosyndactyly , is a branchial arch syndrome, characterized by a number of clinical features, resulting from a developmental anomaly. (the-medical-dictionary.com)
  • PMID- 5428628 TI - The lysosomal nature of the anomalous granules and chromosome aberrations in cultures of peripheral blood in Chediak-Higashi syndrome. (nih.gov)
  • Symptoms of cushing syndrome include a round face, thin arms and legs, severe fatigue and muscle weakness, high blood pressure, high blood sugar, purple or pink stretch marks on the skin, and weight gain, especially in the abdomen. (icd9data.com)
  • Debido a la biosíntesis anormal de esteroides, las características clínicas comprenden VIRILISMO en las mujeres, FEMINIZACIÓN en los varones o desarrollo puberal precoz en los niños.Trastorno de la DIFERENCIACIÓN SEXUAL causado por alteraciones de las GÓNADAS o de las GLÁNDULAS SUPRARRENALES, como la HIPERPLASIA SUPRARRENAL CONGÉNITA y las NEOPLASIAS DE LA CORTEZA SUPRARRENAL. (bvsalud.org)
  • Debido a la biosíntesis anormal de esteroides, los cuadros clínicos pueden ser de VIRILIZACIÓN en las mujeres, de FEMINIZACIÓN en los hombres, o de desarrollo sexual precoz en los niños. (bvsalud.org)
  • Fluoxetine is the presenting symptoms in preceding weeks helpful ambien benadryl in short bowel syndrome. (albionfoundation.org)