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Female pseudohermaphroditism

  The karyotype of female pseudohermaphrodites is 46,XX, the reproductive glands are ovaries, and the internal reproductive organs include the uterus, cervix, and vagina, all of which are present, but the external genitalia show partial masculinization. The degree of masculinization depends on the timing and amount of exposure to high androgens during embryogenesis, ranging from moderately large clitoris to fusion of the labia posteriorly and the appearance of a penis. The cause of high androgens can be congenital adrenal cortical hyperplasia, or it may be from non-adrenal sources.

Table of Contents

1. What are the causes of female pseudohermaphroditism?
2. What complications can female pseudohermaphroditism easily lead to?
3. What are the typical symptoms of female pseudohermaphroditism?
4. How should female pseudohermaphroditism be prevented?
5. What kind of laboratory tests are needed for female pseudohermaphroditism?
6. Diet taboos for patients with female pseudohermaphroditism
7. Conventional methods of Western medicine for the treatment of female pseudohermaphroditism

1. What are the causes of female pseudohermaphroditism?

  Female pseudohermaphroditism is mainly caused by the fetus being exposed to an environment with excessive androgens, and there are three specific causes of the disease:

  1、先天性肾上腺皮质增生,皮质激素合成中21-羟化酶或11-羟化酶缺乏时,出现女性男性化表现。

  1. Congenital adrenal hyperplasia, when there is a deficiency in 21-hydroxylase or 11-hydroxylase in the synthesis of corticosteroids, female masculinization manifestations occur.

  2. The occurrence of masculinization tumors during pregnancy, such as ovarian cysts with excessive secretion of androgens, corpus luteum tumors, ovarian male cell tumors, or benign adrenal tumors. If the mother shows masculinization during pregnancy, the fetus may also have female male external genitalia morphology.

3. The use of androgenic drugs by the mother during pregnancy can also cause masculinization in female fetuses.. 2

  What complications are easily caused by female pseudohermaphroditism

In addition to general symptoms, female pseudohermaphroditism can also cause other diseases. The most common complications in this disease are secondary infertility, amenorrhea, and so on. Therefore, once discovered, active treatment should be carried out, and preventive measures should also be taken in daily life.. 3

  What are the typical symptoms of female pseudohermaphroditism

The clinical symptoms of female pseudohermaphroditism patients include amenorrhea, abnormal development of the external genitalia, indeterminate sex, low voice, short stature, hypertrophy of the clitoris, fusion of the labia minora, and hirsutism. The development of the uterus, ovaries, and vagina is normal.. 4

  How to prevent female pseudohermaphroditism
  Preventive measures for female pseudohermaphroditism:
  1. Perform prenatal diagnosis, chorionic villus sampling: diagnose chromosomal abnormalities of the embryo through chorionic villus biopsy. This examination can be performed at 8 weeks of pregnancy for early diagnosis.

5. What laboratory tests are needed for female pseudohermaphroditism

  Female pseudohermaphroditism can be caused by hereditary feminization or insufficient masculinization when hereditary as a male, or hereditary as a male but insufficient masculinization. The diagnostic steps are as follows:
  1. Medical history and physical examination Firstly, inquire about the patient's mother's history of taking high-efficiency progesterone or danazol drugs during early pregnancy, whether there is a history of similar malformations in the family, and conduct a detailed physical examination. Pay attention to the size of the penis, the position of the urethral opening, and whether there is a vagina and uterus, rectum, and abdominal palpation for the uterus indicates that it is mostly female pseudohermaphroditism, but the possibility of true hermaphroditism should be excluded. If the testes are palpated in the inguinal canal, labia majora, or scrotum, it is undoubtedly testicular tissue, but true hermaphroditism cannot be ruled out.
  2. Laboratory examination For those with a karyotype of 46, XX, low blood estradiol levels, high blood androgen levels, and high levels of urinary 17-ketones and 17α-hydroxypregnenolone, it is congenital adrenal hyperplasia. For those with a karyotype of 46, XY, normal FSH levels, elevated LH levels, and testosterone levels within the normal male range, but estradiol levels higher than normal males but lower than normal females, it is androgen insensitivity syndrome.
  3. Gonadal biopsy The diagnosis of true and false hermaphroditism often requires laparoscopic examination or laparotomy to take a biopsy of the gonads in order to make a final diagnosis.

6. Dietary taboos for female hermaphroditism patients

  The diet of female hermaphroditism patients should be light, with an emphasis on vegetables and fruits, a reasonable diet, and attention to adequate nutrition. The diet of patients should be light and easy to digest, with an emphasis on vegetables and fruits, a reasonable diet, and attention to adequate nutrition. In addition, patients should also pay attention to avoiding spicy, greasy, and cold foods.

7. Conventional Methods of Western Medicine for the Treatment of Female Hermaphroditism

  After clear diagnosis, corrective treatment should be provided according to the patient's original social gender, personal wishes, and the degree of malformation. In principle, for any intersex malformation, except for those with well-developed penises, it is advisable to raise them as females.
  The common treatment methods for intersex disorders are as follows.
  1. Congenital Adrenal Hyperplasia After diagnosis, corticosteroids should be started and given lifelong to suppress the excessive secretion of pituitary adrenocorticotropic hormone and prevent further virilization of the external genitalia and early closure of the epiphyses, which can also promote the development of female reproductive organs and the onset of menstruation, and even there is a possibility of pregnancy and childbirth; the hypertrophied clitoris should be partially excised, and only the clitoral glans should be retained to approach the size of a normal female clitoris; for those with fused malformations in the external genitalia, surgical correction should be performed to expose the urinary meatus and vaginal orifice separately.
  2. Androgen Insensitivity Syndrome It is advisable to raise them as females regardless of complete or incomplete types. Complete patients can wait until they reach puberty and develop into maturity to remove both testicles to prevent malignancy, and long-term estrogen should be given after surgery to maintain female secondary sexual characteristics. Incomplete patients have male external genitalia malformations and should undergo plastic surgery and remove both testicles in advance. Vaginoplasty can be performed for those with short vaginas that hinder sexual life.
  3. Other Male Hermaphroditism Patients with mixed or simple incomplete gonadal development whose karyotypes contain XY have a higher frequency of gonadal malignancy and may occur at a very young age, so the undifferentiated gonads should be removed as soon as possible after diagnosis.
  4. True Hermaphroditism The determination of gender mainly depends on the functional status of the external genitalia. The unnecessary reproductive glands should be removed, and the reproductive glands suitable for the gender should be retained. Generally, in addition to the large penis that can be erect and has testicles that can be pushed into the scrotum, it is advisable to raise them as females. Otherwise, it is better to raise them as females.

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