Article1 May 1961VIRILIZING CONGENITAL ADRENAL HYPERPLASIA: TWENTY-FIVE YEARS LATERJOHN E. JOHNSON JR., M.D.JOHN E. JOHNSON JR., M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-54-5-924 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptOne of the most remarkable advances in clinical endocrinology has been the elucidation of the pathogenesis, clinical features, and treatment of congenital adrenal hyperplasia.1-3In the fetal adrenal cortex of affected persons, synthesis of hydrocortisone is defective owing to a deficiency of specific hydroxylating enzymes. A larger amount of adrenocorticotrophin (ACTH) stimulation of the adrenal is necessary to produce that blood level of hydrocortisone which will in turn suppress and regulate pituitary production of ACTH. Thus, an equilibrium is established at a supranormal level of ACTH release, which causes morphologic hyperplasia of the adrenal cortex. The functional concomitant is increased...Bibliography1. BartterAlbrightForbesLeafDempseyCarroll FCFAPAEE: The effects of adrenocorticotropic hormone and cortisone in the adrenogenital syndrome associated with congenital adrenal hyperplasia: an attempt to explain and correct its disordered hormonal pattern. J. Clin. Invest. 30: 237-251 (March) 1951. CrossrefMedlineGoogle Scholar2. Jailer JW: Virilism. Bull. N. Y. Acad. Med. 27: 377-394 (May) 1953. Google Scholar3. WilkinsBongiovanniClaytonGrumbachVan Wyk LAMGWMMJJ: Virilizing adrenal hyperplasia: its treatment with cortisone and the nature of the steroid abnormalities, in Ciba Foundation Colloquia on Endocrinology, Vol. 8. Little, Brown and Company, Boston, pp. 460-486 (June) 1954. Google Scholar4. Wilkins L: The diagnosis and treatment of endocrine disorders in childhood and adolescence. Charles C Thomas, Springfield, Ill., 1957, pp. 347-348. Google Scholar5. EberleinBongiovanni WRAM: Congenital adrenal hyperplasia with hypertension: unusual steroid pattern in blood and urine. J. Clin. Endocr. 15: 1531-1538 (Dec.) 1955. CrossrefGoogle Scholar6. O'Farrell JM: Hereditary hermaphroditism: report of three cases, J. A. M. A. 104: 1968-1972 (June 1) 1935. CrossrefGoogle Scholar7. JohnsonRemmersClaytonGregory JEARGWR: Brief recording: the short term effect of amphenone in a patient with congenital adrenal hyperplasia, Tex. Rep. Biol. Med., 15: 381-385 (Fall) 1957. MedlineGoogle Scholar8. Young HH: Genital abnormalities, hermaphroditism and related adrenal diseases. The Williams & Wilkins Co., Baltimore, 1937. Google Scholar9. HamwiSerbinKruger GJRAFA: Does adrenocortical hyperplasia result in adrenocortical carcinoma? New Engl. J. Med. 257: 1153-1157, 1957. CrossrefMedlineGoogle Scholar10. ChildsGrumbachVan Wyk BFMMJJ: Virilizing adrenal hyperplasia; a genetic and hormonal study. J. Clin. Invest. 35: 213-223, 1956. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Galveston, Texas*Received for publication October 20, 1960.From the Department of Internal Medicine and the John Sealy Memorial Laboratory for Clinical Research, The University of Texas—Medical Branch, Galveston, Tex.Requests for reprints should be addressed to John E. Johnson, Jr., M.D., Assistant Professor of Medicine, The University of Texas—Medical Branch, Galveston, Tex. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byFertility among women with classical congenital adrenal hyperplasia: Report of seven cases where treatment was started after 9 years of ageAdrenogenital syndrome: absence of the vagina with pregnancy and successful delivery 1 May 1961Volume 54, Issue 5Page: 924-937KeywordsAdrenal cortexAdrenocorticotropic hormoneCongenital adrenal hyperplasiaCorticosteroid therapyEndocrinologyEnzymesExcretionGenital anatomyPathogenesisResearch laboratories Issue Published: 1 May 1961 PDF downloadLoading ...