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PubMedDate not supplied

Laboratory evaluation of the adrenogenital syndrome.

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. 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. Laboratory measurements of hormone metabolites in urine, because of their nonspecificity, lack of sensitivity, and multiple assay steps resulting in poor yield, have been supplanted by specific and sensitive radioimmunoassays of steroid hormones in plasma. In the laboratory evaluation of the adrenogenital syndrome, problems involved in some of the immunoassays of selected hormones should be addressed. Variables owing to specimen collection, storage and handling, and the assay itself should be minimized and controlled.

3-Hydroxysteroid Dehydrogenases↗
PubMed1992

Testicular masses in association with adrenogenital syndrome: US findings.

Adrenogenital syndrome (AGS) is the result of inborn enzymatic defects in the synthesis of steroid hormones. The production of cortisol is deficient and that of adrenocorticotropic hormone is increased. Sometimes male patients have clinically detectable testicular lesions, known as testicular tumors of AGS (TTAGS). From 1985 to 1991, scrotal ultrasonography (US) was performed in 30 consecutive pubertal and postpubertal patients with AGS to investigate the prevalence and US characteristics of TTAGS. Eight of 30 patients had a testicular lesion (27%); six of the eight lesions were clinically undetected. The mean diameter of the lesions was 16.44 mm (range, 2-28 mm). The lesions were hypoechoic in all cases, with well-defined margins in six cases. The nodules were multifocal in all patients and bilateral in six (75%). If testicular lesions are present in a patient with AGS, TTAGS are likely, and frequent US monitoring is adequate for diagnostic evaluation.

Adolescent↗
PubMed2002

[Adrenogenital syndrome: feminizing genital reconstruction].

Adrenogenital syndrome, or so called congenital adrenal hyperplasia, is caused by a congenital insufficiency of the enzyme 21-hydroxylase, which is responsible for converting cortisol into cholesterol. Because of virilizing effect of androgens overproduction girls develop clitoral hypertrophy and persistent urogenital sinus (common channel for urethra and vagina). Surgical treatment is recommended in order to repair those developmental faults. The aim of this study was to employ the contemporary surgical techniques and to evaluate the postoperative results. Forty-seven patients affected by adrenogenital syndrome were investigated and treated at two institutions: Departments of Pediatric Surgery of Copenhagen University Hospital and Kaunas Medical University Hospital. Forty-three patients have been operated and underwent genitoplasty. Surgical method was chosen individually depending on the height of the urogenital sinus. In a case of low sinus a simple cut-back procedure was performed. In a case of high sinus the more complex procedure such as total urogenital mobilization or vaginal pull through would be involved. All patients underwent vaginal dilatations for 6-12 months postoperatively. Twenty-eight patients underwent clitoroplasty while the glans and the neurovascular bundle were preserved and clitoral skin used for plasty of the labia minora. Postoperatively the patients were observed for 0.5-5 years, the close results showed to be good. There were 4 cases of vaginal stenosis and 2 cases of urethrovaginal fistula (all successfully repaired later). Early one staged genitoplasty and postoperative vaginal dilatations for the period of 6-12 months is recommended.

Adolescent↗
PubMed2003

[Bilateral testicular masses in the scope of adrenogenital syndrome].

Testicular masses in male individuals with the adrenogenital syndrome (AGS) are a clinical and pathological diagnostic dilemma. The major differential diagnosis of gonadal nodules in this setting includes interstitial Leydig cell tumors and secondary benign tumors possibly of adrenal origin. We report a case of adrenogenital syndrome occurring in a 14-year-old boy. Examinations to clarify the cause of his dwarfism and bilateral testicular masses revealed 21-hydroxylase deficiency. The testes were not tender and were firm and nodular on palpation. The serum levels of adrenocorticotrophic hormone (ACTH), 17 alpha-hydroxyprogesterone (17-alpha-OHP), testosterone, and aldosterone were found to be elevated. Under corticosteroid therapy the serum marker abnormalities were corrected and there was gradual regression of the tumor lesions in both testes. Testicular tumors with adrenogenital syndrome are typically bilateral and develop in untreated or inadequately treated males with AGS.

Adolescent↗