Hypogonadotrophic Hypogonadism Simulating Turner's Syndrome

Case Reports1 September 1961Hypogonadotrophic Hypogonadism Simulating Turner's SyndromeABNER J. DELMAN, M.D., JEROME G. PORUSH, M.D., ALVIN D. SCHWARTZ, M.D.ABNER J. DELMAN, M.D.Search for more papers by this author, JEROME G. PORUSH, M.D.Search for more papers by this author, ALVIN D. SCHWARTZ, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-55-3-471 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe clinical manifestations of panhypopituitarism secondary to destruction of the adenohypophysis are well known. After its original description by Simmonds (1) in 1914, panhypopituitarism was the subject of many case reports and reviews (2-5). Marked insufficiency of the target glands and profound cachexia were stressed. Sheehan and Murdoch (6, 7) first emphasized that most patients with pituitary insufficiency are not cachetic and that there is a wide clinical spectrum of pituitary dysfunction. Hypopituitarism may vary from mild involvement of one or more of the target glands to severe insufficiency of all three principal organs (6-8). The clinical course may range...References1. SIMMONDS M: Ueber Hypophysesschwiend mit todlichen augang. Deutsch. Med. Wschr. 40: 322, 1914. CrossrefGoogle Scholar2. CALDER RM: Anterior pituitary insufficiency (Simmonds' disease). Bull. Johns Hopkins Hosp. 50: 87, 1932. Google Scholar3. SILVER S: Simmonds' disease. Arch. Intern. Med. 51: 175, 1933. CrossrefGoogle Scholar4. HERRICK WW: Clinical features of hypophyseal cachexia. Ass. Res. Nerv. Dis. Proc. 17: 609, 1938. Google Scholar5. ESCAMILLALISSER RFH: Simmonds' disease. J. Clin. Endocr. 2: 65, 1942. CrossrefGoogle Scholar6. SHEEHAN HL: Post partum necrosis of anterior pituitary. Lancet 1: 817, 1939. Google Scholar7. SHEEHANMURDOCH HLL: Simmonds' disease due to post partum necrosis of the anterior pituitary. Quart. J. Med. 32: 277, 1939. Google Scholar8. SHEEHANSUMMERS HLOW: Syndrome of hypopituitarism. Quart. J. Med. 18: 319, 1949. MedlineGoogle Scholar9. HUBBLE D: The course of anterior hypopituitarism. Lancet 1: 1123, 1952. CrossrefMedlineGoogle Scholar10. SOFFER LJ: Diseases of Endocrine Glands, Lea & Febiger, Philadelphia, 1956. Google Scholar11. EDITORIAL: Selective pituitary failure. Lancet 1: 556, 1954. Google Scholar12. OELBAUM MH(a) : Variability of endocrine dysfunction in postpartum hypopituitarism. Brit. Med. J. 2: 110, 1952. (b) WILSON, L. A., AULD, W. H., BOWMAN, W.: Incomplete pituitary insufficiency. Lancet 2: 715, 1954. (c) PASCHKIS, K. E., CANTAROW, A.: Hypopituitarism: studies in pituitary tumors and Simmonds' disease. Ann. Intern. Med. 34: 669, 1951. CrossrefMedlineGoogle Scholar13. ALBRIGHTHALSTED FJA: Studies in ovarian dysfunction. II. Application of the 'hormonal measuring sticks' to the sorting out and to the therapy of various types of amenorrhea. New Engl. J. Med. 212: 250, 1935. CrossrefGoogle Scholar14. FRASERFORBESALBRIGHTSULKOWITCHREIFENSTEIN RWAPFHEC: Colorometric assay of 17-ketosteroids in the urine; survey of the use of this test in endocrine investigation, diagnosis and therapy. J. Clin. Endocr. 1: 234, 1941. CrossrefGoogle Scholar15. KLINEFELTERALBRIGHTGRISWOLD HFFGC: Experience with a quantitative test for normal or decreased amounts of gonadotrophin in endocrinological diseases. J. Clin. Endocr. 3: 529, 1943. CrossrefGoogle Scholar16. WILKINSFLEISCHMANN LW: Ovarian agenesis: pathology, associated clinical symptoms, bearing on theories of sex differentiation. J. Clin. Endocr. 4: 357, 1944. CrossrefGoogle Scholar17. HOWARDSNIFFENSIMMONSALBRIGHT RPRCFAF(a) : Testicular deficiency: clinical and pathologic study. J. Clin. Endocr. 10: 121, 1950. (b) HELLER, C. G., NELSON, W. O.: Classification of male hypogonadism, pathologic physiology, diagnosis and treatment. J. Clin. Endocr. 8: 345, 1948. CrossrefGoogle Scholar18. TURNER HH: Syndrome of infantilism, congenital webbed neck and cubitus valgus. Endocrinology 23: 566, 1938. CrossrefGoogle Scholar19. ALBRIGHTSMITHFRASER FPHR: A syndrome characterized by primary ovarian insufficiency and decreased stature. Amer. J. Med. Sci. 204: 625, 1942. CrossrefGoogle Scholar20. DEL CASTILLODE LA BALZEARGONZ EBFAJ: Syndrome of rudimentary ovaries with estrogenic insufficiency and elevated gonadotrophins. J. Clin. Endocr. 7: 385, 1947. CrossrefGoogle Scholar21. LISSERCURTISESCAMILLAGOLDBERG HLERFMB: Syndrome of congenitally aplastic ovaries with sexual infantilism, high urinary gonadotrophins, short stature and other congenital abnormalities. J. Clin. Endocr. 7: 665, 1947. CrossrefGoogle Scholar22. PASCHKISRAKOFFCANTAROW KEAEA: Clinical Endocrinology, Paul B. Hoeber, Inc., New York, 1958. Google Scholar23. JACKSONSORIGEN WPR: Turner's syndrome in the female. Brit. Med. J. 2: 368, 1953. CrossrefMedlineGoogle Scholar24. HOFFENBERGJACKSON RWP(a) : Gonadal dysgenesis: modern concepts. Brit. Med. J. 2: 1457, 1957. (b) WILKINS, L., GRUMBACH, M. M., VAN WYK, J. J.: Chromosomal sex in 'ovarian agenesis.' J. Clin. Endocr. 14: 1270, 1954. CrossrefMedlineGoogle Scholar25. SILVER HK(a) : Syndrome of ovarian agenesis. Pediatrics 8: 368, 1951. (b) VARNEY, R. F., KENYON, A. T., KOCH, F. C.: Association of short stature, retarded sexual development and high urinary gonadotrophin titers in women. J. Clin. Endocr. 2: 137, 1942. MedlineGoogle Scholar26. DORFFAPPELMANLIVESON GBDHA: Report on a case showing congenital defects, short stature, retarded sexual development and no urinary gonadotrophins. J. Clin. Endocr. 7: 807, 1947. CrossrefGoogle Scholar27. HERTZCROMERWESTFALL RJKBB: Case of ovarian agenesis with normal urinary gonadotrophin titer. J. Clin. Endocr. 10: 610, 1950. CrossrefGoogle Scholar28. CARPENTIERSTOLTEVISSCHERS JJLAGP: Determination of genetic sex by vaginal smear. J. Clin. Endocr. 16: 155, 1956. CrossrefGoogle Scholar29. GREENBLATTCARMONA RBN: The eunuchoid female—a case study. J. Clin. Endocr. 15: 877, 1955. Google Scholar30. BRADBURYBROWNBROWN JTESWE: Adsorption of urinary gonadotrophins on kaolin. Proc. Soc. Exp. Biol. Med. 71: 228, 1949. CrossrefMedlineGoogle Scholar31. PURVEEGRISBACH KEWE: Special staining studies of the rat pituitary gland. Endocrinology 49: 244, 1951. MedlineGoogle Scholar32. MOLDAWERALBRIGHTBENEDICTFORBESHENNEMAN MPFPHAPPH: Eunuchoidism with low urinary FSH in the female: comparison with this syndrome in the male and with pre-menarchal menopause. J. Clin. Endocr. 18: 1, 1958. CrossrefGoogle Scholar33. FERGUSON-SMITH MA(a) : Cytogenetics in man. Arch. Intern. Med. 105: 627, 1960. (b) MOORE, K. L., GRAHAM, M. A., BARR, M. L.: The detection of chromosome sex in hermaphrodites from a skin biopsy. Surg. Gynec. Obstet. 96: 641, 1953. (c) GRUMBACH, M. M., BARR, M. L.: Cytologic tests of chromosomal sex in relation to sexual anomalies in man. Recent Progr. Hormone Res. 14: 255, 1958. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: New York, N. Y.Received for publication August 17, 1960.From the Departments of Medicine and Endocrinology, The Mount Sinai Hospital, New York, N. Y.Requests for reprints should be addressed to Abner J. Delman, M.D., Montefiore Hospital, 210th Street and Bainbridge Avenue, New York 67, N. Y. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byHypogonadotropic Hypogonadism Associated with Anosmia in the Female 1 September 1961Volume 55, Issue 3Page: 471-478KeywordsEndocrinologyHospital medicineHypogonadismPanhypopituitarismTurner syndrome Issue Published: 1 September 1961 PDF downloadLoading ...