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[Pseudohermaphroditism due to XY gonadal absence syndrome (author's transl)].

作者信息

Mühlenstedt D, Schulte M J

出版信息

Arch Gynakol. 1977 Oct 28;223(3):241-7. doi: 10.1007/BF00667394.

DOI:10.1007/BF00667394
PMID:579301
Abstract

A case about a 16 year old individual is presented, in whom no internal genitals and vagina were found; no signs of breast development, axillary and pubic hair growth could be detected. The external genitals were hypoplastic. A sinus urogenitalis was present. The sex chromosome pattern was normal male (XY). On both sides of the pelvic wall rudiments of the Fallopian tube were observed by laparoscopy. Testosterone, estradiol, 17 alpha-OH-progesterone as well as androstendione-secretion could not be stimulated by HCG. Basal and LH-RH stimulated gonadotropins indicated a hypergonadotropic state. Height and bodyweight were in the range of a 12-year old boy, the bone age correlated with 14 years.

摘要

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引用本文的文献

1
Sexual differentiation of the hypothalamus in gonadal agenesis and testicular feminization.
Arch Gynecol. 1979 Aug;227(2):97-102. doi: 10.1007/BF02103283.

本文引用的文献

1
AN XY FEMALE WITH ABSENT GONADS AND VESTIGIAL PELVIC ORGANS.
J Obstet Gynaecol Br Commonw. 1963 Aug;70:675-80. doi: 10.1111/j.1471-0528.1963.tb04966.x.
2
[Case of gonad dysgenesis with adrenogenic manifestations].
Arch Fr Pediatr. 1958;15(5):613-22.
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True agonadism (anorchism) in siblings.
Gynaecologia. 1956 Oct;142(4):215-33.
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[Malformations of the gonad].
Dtsch Med Wochenschr. 1956 Aug 17;81(33):1298-302. doi: 10.1055/s-0028-1115119.
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Pseudohermaphroditism with multiple congenital anomalies; report of a case.
Pediatrics. 1955 Sep;16(3):363-71.
6
Concomitant malformations; the frequent, simultaneous occurrence of congenital malformations of the reproductive and urinary tracts.合并畸形；生殖和泌尿系统先天性畸形的频繁同时发生。
Obstet Gynecol. 1953 Sep;2(3):236-65.
7
[Agonadism (anorchism) in a 6-year-old child].[一名6岁儿童的无性腺症（无睾症）]
Dtsch Med Wochenschr. 1968 Mar 29;93(13):633-9. doi: 10.1055/s-0028-1105108.
8
Agonadism.无性腺症
Can Med Assoc J. 1965 Nov 13;93(20):1080-3.
9
The XY gonadal agenesis syndrome.
J Med Genet. 1973 Sep;10(3):288-93. doi: 10.1136/jmg.10.3.288.
10
Endocrine and metabolic studies in an XY patient with gonadal agenesis.
J Clin Endocrinol Metab. 1974 Sep;39(3):540-7. doi: 10.1210/jcem-39-3-540.