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Syndrome Characterized by Gynecomastia, Aspermatogenesis without A-Leydigism, and Increased Excretion of Follicle-Stimulating Hormone1

Published on Nov 1, 1942in The Journal of Clinical Endocrinology and Metabolism5.605
· DOI :10.1210/jcem-2-11-615
Harry F. Klinefelter2
Estimated H-index: 2
(Harvard University),
Edward C. Reifenstein12
Estimated H-index: 12
(Harvard University),
Fuller Albright48
Estimated H-index: 48
(Harvard University)
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Abstract
The syndrome under discussion begins during 11 adolescence and is characterised by gynecomastia and a very specific type of hypogonadism. This latter is almost entirely in respect to the function of the tubular tissue (germinal epithelium and Sertoli cells) while the function of the Leydig cells (growth of phallus and prostate and of sexual hair) remains relatively normal. Thus one finds bilateral gynecomastia, small testes, aspermatogenesis. evidence of normal to moderately reduced function of the Leydig cells, increased excretion of follicle-stimulating hormone (FSH), and usually a reduced excretion of 17-ketosteroids. During the last 4 years, 7 cases have been observed in the clinics of the Massachusetts General Hospital; studies on these patients and two additional private patients form the subject of this report. review of literature Although these cases are not uncommon, few reports are found in the literature, and to our knowledge, no author has grouped them together as a definite clinical entity.
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#1Margo Willems (Vrije Universiteit Brussel)
#2Inge GiesH-Index: 9
Last. Dorien Van Saen (Vrije Universiteit Brussel)H-Index: 7
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#1M. Spaziani (Sapienza University of Rome)H-Index: 3
#2S. Granato (Sapienza University of Rome)H-Index: 3
Last. Andrea Lenzi (Sapienza University of Rome)H-Index: 57
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PURPOSE: Klinefelter syndrome (KS) is a genetic disorder caused by the presence of an extra X chromosome in males. The aim of this study was to evaluate the hypothalamic-pituitary-gonadal (HPG) axis and the clinical profile of KS boys from mini-puberty to early childhood. PATIENTS AND METHODS: In this retrospective, cross-sectional, population study, 145 KS boys and 97 controls aged 0-11.9 years were recruited. Serum FSH, LH, testosterone (T), Inhibin B (INHB), sex hormone binding globulin (SHBG...
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#1Carole A. Samango-Sprouse (FIU: Florida International University)H-Index: 18
Last. Andrea L. Gropman (GW: George Washington University)H-Index: 36
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This cross-sectional study examined the neurodevelopment of a large, prenatally diagnosed population of boys with 47,XXY; investigated the potentially positive effects of early hormonal therapy (EHT) on language, cognition, and motor in this population; and identified novel at risk biomarkers associated with 47,XXY. Two-hundred and seventy two evaluations were collected from 148 prenatally diagnosed boys with 47,XXY between 0 and 36 months and separated into one of three groups, depending on vis...
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#2Jingtao GuoH-Index: 2
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#1Divya Namboodiri (Macquarie University)
#2Hui Yi Ng (Macquarie University)
Last. Veronica Preda (Macquarie University)
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Gonadotropinoma in Klinefelter syndrome (KS) is uncommon and may be a result of protracted stimulation of gonadotrophs from lack of androgen feedback. Associations of white matter changes and increased venous thromboembolic risk have been reported and need to be considered in patients with KS.
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#1Kenta Saito (Fujita Health University)H-Index: 1
#2Yohei Iwata (Fujita Health University)H-Index: 7
Last. Kazumitsu Sugiura (Fujita Health University)H-Index: 25
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#1Hisanori Taniguchi (Kansai Medical University)H-Index: 4
#2MatsudaTadashi (Kansai Medical University)H-Index: 21
Last. Yoshiharu MorimotoH-Index: 15
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Abstract Introduction For couples who wish for a baby, sexual activity (through intercourse) is the only way to conceive naturally. However, the sexual activity of men undergoing testicular sperm extraction (TESE) and factors that influence it among affected couples are not clearly understood. Aim To examine sexual activity among infertile men undergoing TESE and to identify factors that influenced it. Methods Married Japanese male patients undergoing TESE were included. Sexual activity during t...
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#1Darshan P. Patel (UofU: University of Utah)H-Index: 8
#2Timothy G. Jenkins (BYU: Brigham Young University)
Last. James M. Hotaling (UofU: University of Utah)H-Index: 27
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The complexity of male reproductive impairment has hampered characterization of the underlying genetic causes of male infertility. However, in the last 20 years, more powerful and affordable tools to interrogate the genetic and epigenetic determinants of male infertility have accelerated the number of new discoveries in the characterization of male infertility. With this explosion of new data, integration in a systems-based approach—including complete phenotypic information—to male infertility i...
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#1Noritoshi EnatsuH-Index: 5
#2Yihsien EnatsuH-Index: 1
Last. Masahide ShiotaniH-Index: 14
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A 27-year-old man with nonobstructive azoospermia was diagnosed with Klinefelter syndrome (KS) with a satellite Y chromosome (47, XXYqs) by karyotyping. Genetic analysis revealed azoospermia factor c (AZFc) microdeletion of gr/gr deletion in the Y chromosome. Microdissection testicular sperm extraction (micro-TESE) was performed in bilateral testes. Very few seminiferous tubules were bilaterally observed, and a minute number of spermatozoa obtained from the left testis were cryopreserved. Histol...
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