Abstract IN 1938 HENRY TURNER1 reported a syndrome consisting of shortness of stature, sexual underdevelopment, short, "webbed" neck, and cubitus valgus, features he found in seven girls, aged 15 to 23. In his opinion this syndrome was brought about by pituitary insufficiency. It should be mentioned, however, that in 1930 Ulrich2 had already reported a somewhat similar "pterygium syndrome." Later, Varney et al3 and Albright et al4 proved that the cause of sexual underdevelopment in such cases lies in gonadal aplasia or dysgenesis with a secondary rise of urinary gonadotropins. Polani5 showed that in 80% of these cases sex chromatin was negative (male) and the chromosome constition was most often XO. Isolated sexual underdevelopment is now called "pure" gonadal dysgenesis. On the other hand, gonadal dysgenesis combined with some characteristic anomalies of other organs is usually called Turner's syndrome, formerly also Ulrich-Turner's syndrome. Here the following References 1. Turner, H.: A Syndrome of Infantilism, Congenital Webbed Neck and Cubitus Valgus , Endocrinology 23:566-574, 1938.Crossref 2. Ulrich, O.: Über typische Kombinationsbilder multipler Abartungen , Z Kinderheilk 49:271, 1930.Crossref 3. Varney, R.F.; Kenyon, A.T.; and Koch, F.C.: Association of Short Stature, Retarded Sexual Development and High Urinary Gonadotropin Titers in Women: Ovarian Dwarfism , J Clin Endocr 2:137-145, 1942.Crossref 4. Albright, E.; Smith, P.H.; and Fraser, R.: A Syndrome Characterized by Primary Ovarian Insufficiency and Decreased Stature , Amer J Med Sci 204:625-648, 1942.Crossref 5. Polani, P.E.; Lessof, M.H.; and Bishop, P.M.: Colour Blindness in "Ovarian Agenesis," Lancet 2:118-120 ( (July 21) ) 1956.Crossref 6. Ferrier, P.: Cytogenetique de la differentiation sexuelle chez l'homme , J Génét Hum 14:53, 1965. 7. Stratton, H.: Gonadal Dysgenesis and the Ears , J Laryng 79:343 (No. (4) ) 1965.Crossref 8. Lindsten, J., and Tillinger, K.: Cytogenetical and Clinical Investigations in Patients With Turner's Syndrome , Endokr Pol 16:141-149 ( (March) April) 1965. 9. Partsch, C., and Schmidt-Wittkamp, E.: Pterygium-Syndrom und Schwerhörigkeit , Arch Ohr Nas Kehlkopfheilk 183:336, 1964.Crossref 10. Lenz, W.: Störungne der primären Geschlechtsentwicklung , Mschr Kinderheilk 109:131-139 ( (March) ) 1961. 11. Diamant, M.: The "Pathologic Size" of the Mastoid Cell System , Acta Otolaryng 60:1-10 ( (July) Aug) 1965.Crossref 12. Albrecht, W.: Pneumatisation und Konstitution , Z Ohr Nas Kehlkopfheilk 10:51, 1924. 13. Schwarz, M.: Die Bedeutung der hereditären Anlage für die Pneumatisation der Warzenfortsätze und der Nasennebenhöhlen , Arch Ohr Nas Kehlkopf 123:161, 1929.Crossref 14. Diamant, M.: Studies on Causative Factors of Mastoid Pneumatization , Arch Otolaryng 68:587 (No. (5) ) 1958.Crossref 15. Flisberg, K.; Ingelstedt, S.; and Örtegren, U.: The Relationship of Middle Ear Disease to Mastoid Hypocellularity , Atca Otolaryng (suppl 182) , p 69, 1963.
Archives of Otolaryngology – American Medical Association
Published: Jan 1, 1968
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