Adrenogenital Syndrome

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.

Also Known As:

Networked: 273 relevant articles (0 outcomes, 12 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Hyperplasia
2.	Cushing Syndrome
3.	Pituitary ACTH Hypersecretion
4.	Carcinoma (Carcinomatosis)
5.	Phenylketonurias (Phenylketonuria)

Experts

1.	Ulbright, Thomas M: 3 articles (09/2014 - 02/2005)
2.	Kim, Kyu-Rae: 2 articles (02/2022 - 04/2004)
3.	Young, Robert H: 2 articles (09/2014 - 09/2014)
4.	Khanna, A: 2 articles (03/2006 - 04/2001)
5.	Kurtzman, N A: 2 articles (03/2006 - 04/2001)
6.	Park, Nora Jee-Young: 1 article (02/2022)
7.	Yoo, Su Hyun: 1 article (02/2022)
8.	Bordier, Lyse: 1 article (01/2021)
9.	Chiron, Paul: 1 article (01/2021)
10.	Dusaud, Marie: 1 article (01/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Adrenogenital Syndrome:

1.	SteroidsIBA 01/01/1984 - "An in vitro study has been made of the steroid metabolism in the abdominal skin in two hirsute women with adrenogenital syndrome, and the concentrations of the various steroids in the skin tissue have been determined. " 04/01/1954 - "Studies on the metabolism of adrenal steroids in the adrenogenital syndrome." 03/01/1954 - "The adrenogenital syndrome in children a clinical and steroid metabolic study." 01/03/1997 - "[Diagnosis of the adrenogenital syndrome caused by 11beta-hydroxylase deficiency using gas chromatographic-mass spectrometric analysis of the urinary steroid profile]." 08/01/1977 - "[Urinary excretion of steroids in congenital adrenogenital syndrome with successful pregnancy (author's transl)]."
2.	Hydrocortisone (Cortisol)FDA LinkGeneric 04/25/1980 - "Measurement of serum 17-OHP diurnal profile is thus advantageous in treatment of adrenogenital syndrome with cortisol when compared to values obtained from 24-hour urine collections." 06/01/1972 - "Insulin secretion in children with adrenogenital syndrome treated with low doses of oral hydrocortisone." 11/01/1961 - "Adrenogenital syndrome due to enzymatic defects in cortisol synthesis." 07/01/1973 - "[Hydrocortisone therapy of the AGS (adrenogenital syndrome)]." 07/01/1972 - "Cortisol production by testicular tumors in adrenogenital syndrome."
3.	SaltsIBA 08/01/1968 - "These studies provide additional evidence in support of the hypothesis that the salt-losing and "non-salt-losing" forms of adrenogenital syndrome are genetically and biochemically distinct." 01/01/2016 - "The consequences of adrenocortical dysfunction during early development are also recognised in the congenital salt wasting and adrenogenital syndromes presenting neonatally, yet despite a remit to focus on developmental and reproductive toxicity mechanisms of endocrine disruption by many regulatory agencies (USEPA EDSTAC; REACH) the assessment of adrenocortical function has largely been ignored. " 11/01/1986 - "The adrenals and kidney incretory structures (the juxtaglomerular apparatuses and renomedullary interstitial cells) were studied in 12 cases of a desalinization form of the adrenogenital syndrome in children who died at the age of 1-6 mos of acute water-electrolytic disturbances in order to assess function of the structures involved in the regulation of the water-salt equilibrium. " 11/01/1986 - "[Functional morphology of the adrenal cortex and incretory kidney structures in the salt-losing form of adrenogenital syndrome in children]." 01/01/1986 - "Adrenals were studied in 17 children with adrenogenital syndrome with a salt loss. "
4.	AndrogensIBA 07/01/1948 - "A study of the biological activity and the magnitude of endogenous androgen production in a case of adrenogenital syndrome." 01/01/2000 - "To evaluate the urodynamic findings and clinical symptoms in a group of intersex patients and to determine how androgens influence bladder function in female children, clinical and urodynamic records of 12 intersex patients with adrenogenital syndrome were investigated retrospectively. " 07/01/1987 - "To discover whether excessive androgen activity in females might produce ovarian descent, the position of the ovaries in 15 children with severe adrenogenital syndrome was observed. " 10/01/1986 - "Two cases of androgen producing ovarial tumours and one case of adrenogenital syndrome were analysed separately." 05/01/1981 - "The adrenogenital syndrome resulting from excess androgen production occurs as masculinization in the female and precocious maturation in the male. "
5.	Testosterone (Sustanon)FDA Link 05/01/1983 - "The investigation of the plasma testosterone level in the parents of children with the adrenogenital syndrome has shown that an increase in the blood circulating testosterone level 1.6 to 4 times compared with normal may serve as a marker of heterozygosis for this disease." 05/01/1967 - "[A case of adrenogenital syndrome with markedly increased secretion of testosterone]." 07/01/1980 - "The testosterone concentration proved to depend on clinical manifestations in boys with hyper- and hypogonadism and in girls with adrenogenital syndrome. " 06/01/1964 - "[ADRENOGENITAL SYNDROME CAUSED BY CONGENITAL HYPERPLASIA OF THE ADRENAL CORTEX: PATHOGENETIC IMPORTANCE OF TESTOSTERONE]." 07/01/1980 - "The blood plasma testosterone content was determined by radioimmunological method in patients with hirsutism, premature sexual maturation, false male hermaphroditism, true hermaphroditism, mixed gonad dysgenesis, adrenogenital syndrome, and also in some mothers of patients suffering from intersexualism and in healthy persons. "
6.	Adrenal Cortex Hormones (Corticosteroids)IBA 01/01/1967 - "[Studies on corticosteroid biosynthesis in 3 sisters with congenital adrenogenital syndrome]." 07/01/1966 - "[Histomorphological findings in the adrenal cortex in congenital adrenogenital syndrome and their change after corticoid treatment]." 08/01/1960 - "Differences in corticosteroid excretion pattern between infants and children with the adrenogenital syndrome." 04/01/1959 - "The corticosteroid excretion pattern in infants and children with the adrenogenital syndrome." 09/01/2008 - "A 38-year-old female suffered from adrenogenital syndrome with 21-hydroxylase deficiency requiring a corticosteroid substitution. "
7.	17-alpha-Hydroxyprogesterone (17 Hydroxyprogesterone)IBA 12/09/1988 - "The salivary concentration of 17-hydroxyprogesterone (17-OHP) was determined in 11 patients (aged 6 months to 13 years) with congenital adrenogenital syndrome (AGS) due to C21-hydroxylase deficiency and six healthy controls, in order to assess its value in monitoring treatment of AGS. " 09/01/1983 - "[Determination of 17-hydroxyprogesterone levels in the treatment of children with the adrenogenital syndrome]." 07/01/1982 - "[17-hydroxyprogesterone in the diagnosis and treatment of adrenogenital syndrome]." 07/01/1974 - "[Diagnosis and course control of the adrenogenital syndrome of the 21-hydroxylase deficiency type using radioimmunologic demonstration of 17-alpha-hydroxyprogesterone]." 09/01/1956 - "17-Alpha-hydroxyprogesterone and the virilism of the adrenogenital syndrome associated with congenital adrenocortical hyperplasia; a review."
8.	17-Ketosteroids (17 Ketosteroids)IBA 08/25/1960 - "[Chromatography of the 17-ketosteroids and determination of pregnantriol in the adrenogenital syndrome]." 01/14/1957 - "[Absence of urogenital sinus and chromatography of urinary 17-ketosteroids in adrenogenital syndrome; follow-up of a case]." 04/01/1955 - "Urinary excretion of 17-ketosteroids in patients with adrenogenital syndrome." 01/01/1964 - "[Sterility caused by adrenogenital syndrome in a male with azoospermia and high levels of 17-ketosteroids. " 01/01/1969 - "[Study of cortisol precursos (21-deoxysteroids) and the 17-ketosteroids in congenital adrenogenital syndrome]."
9.	EnzymesIBA 01/01/1957 - "[Clinical studies on the function of the enzyme system in the adrenal cortex in adrenal cortical hyperplasia associated with adrenogenital syndrome]." 03/01/1985 - "Laboratory evaluation of the adrenogenital syndrome involves measurement of hormones and metabolites accumulated prior to the enzymic block as well as hormones whose synthesis is affected by deficiency of a specific enzyme. " 03/01/1985 - "The adrenogenital syndrome is a result of the deficiency of one of the enzymes involved in the pathway leading to the synthesis of cortisol by the adrenal cortex. " 08/01/1989 - "The adrenogenital syndrome (AGS) is a relatively common inherited metabolic disease, generally caused by a deficiency of the adrenocortical enzyme steroid 21-hydroxylase. " 01/01/2018 - "The adrenogenital syndrome is an autosomal recessive disorder in which an enzyme defect in the steroid pathway leads to excessive prenatal exposure of androgens. "
10.	ProgesteroneFDA LinkGeneric 01/01/1960 - "[Demonstration of progesterone and 17alpha-hydroxyprogesterone in hyperplastic adrenal glands in the adrenogenital syndrome]." 01/01/1998 - "The involved androgen comes from external factors, a it occurs at mother who receives progesterone to avoid abortion, but it is due frequently to androgen storage, caused by enzymatic blockade at steroidogenesis (adrenogenital syndrome)." 06/01/1982 - "[Significance of progesterone, testosterone and cortisol in the diagnosis of adrenogenital syndrome in children]." 07/20/1984 - "In the present study, the effects of o,p'-DDD on plasma levels of pregnenolone, 17 alpha-hydroxypregnenolone, progesterone, 17 alpha-hydroxyprogesterone, 11-deoxycorticosterone, deoxycortisol, corticosterone, cortisol, androstenedione and testosterone were studied in 6 patients with adrenal carcinoma (3 with Cushing's syndrome, 2 with adrenogenital syndrome, one without clinical manifestation) and 6 with Cushing's disease. "

Therapies and Procedures

1.	Nursing Care 05/27/1976 - "Nursing care study: clitoroplasty for adrenogenital syndrome."
2.	Therapeutics 10/01/2003 - "[Postnatal diagnosis and therapy of adrenogenital syndrome]." 10/01/2003 - "[Adrenogenital syndrome: prenatal diagnosis and therapy]." 03/01/1990 - "[A change in substitution therapy in patients with congenital adrenogenital syndrome within the scope of surgical interventions]." 10/30/1988 - "[Prenatal therapy of adrenogenital syndrome]." 04/01/1987 - "[Medical therapy of the congenital adrenogenital syndrome]."
3.	Adrenalectomy 06/01/1989 - "[Adrenalectomy as therapy in refractory adrenogenital syndrome]." 09/01/1951 - "[Adrenogenital syndrome and sterility; adrenalectomy and subsequent pregnancy]." 01/01/2002 - "Laparoscopic adrenalectomy was performed in 216 patients with a variety of adrenal disorders, including 66 patients with Conn's syndrome, 55 with incidentaloma, 58 with Cushing's syndrome, 33 with pheochromocytoma, two with virilizing adrenogenital syndrome, and two with other lesions. " 11/01/1994 - "From 1982 to 1992, 33 patients underwent adrenalectomy for disease of the adrenal gland: 18 patients with adrenal cortex adenoma or hyperplasia (Cushing's syndrome n = 11, Conn's syndrome n = 6, adrenogenital syndrome n = 1), 3 with pituitary-dependent Cushing's disease, 7 with pheochromocytoma (malignant n = 1), 2 with a metastasis of lung cancer and 1 with cystic adrenal necrosis. " 01/01/1955 - "Cortisone and adrenalectomy in treatment of sterility in women with adrenogenital syndrome."
4.	Orchiectomy (Orchidectomy) 01/01/2016 - "The authors report a case of bilateral Leydig-cell tumour associated with giant bilateral adrenal myelolipoma in a male with adrenogenital syndrome who was submitted to a bilateral orchiectomy. " 02/01/2007 - "Testicular tumor of the adrenogenital syndrome was managed by tumor enucleation in 7 patients (54%) and by radical orchiectomy in 6 (46%). " 07/01/1979 - "In the adult patient inguinal orchiectomy is the treatment of choice, while in the patient with adrenogenital syndrome initial management by high-dose steroid suppression should be attempted prior to testicular exploration." 04/01/1997 - "Surgical management of steroid unresponsive testicular tumors of the adrenogenital syndrome has been orchiectomy. "
5.	Female Circumcision (Infibulation) 01/01/1970 - "Technique of vaginoplasty and clitoridectomy in the adrenogenital syndrome." 01/01/1981 - "Clitoridectomy or plastic reduction of the clitoris in the adrenogenital syndrome."