Non-adrenal hyperplastic pseudohermaphroditism

　　The occurrence of masculinization during pregnancy induced by sex hormones is related to the following factors: the type and dosage of sex hormones; the timing of pregnancy; the permeability and metabolic function of the placenta; the metabolic and degradation function of exogenous hormones in pregnant women; as well as the sensitivity and metabolic dysfunction of the fetal reproductive system to sex hormones.

　　The progesterone taken for threatened abortion is mostly artificial preparations, and its biological action characteristics not only have the effect of progesterone but also androgen, and the synthetic progesterone is more than 10 times stronger than that of natural progesterone, with slow excretion and long action time, thus easily leading to male feminization of female fetuses. The feminization of female fetuses caused by estrogen is due to the metabolic disorder of progesterone produced by synthetic estrogen, as well as the stimulation of the fetal adrenal cortex by synthetic estrogen, promoting the secretion of androgens.

　　Common complications caused by non-adrenal medullary hyperplastic pseudohermaphroditism include:

　　1, Female fetus masculinization:Due to the action of estrogen and progesterone, and the synthetic action of progesterone is more than 10 times stronger than that of natural progesterone, its excretion is slow, and its action time is long, leading to male feminization of female fetuses.

　　2, Endocrine system diseases:Syndromes caused by abnormal secretion function and (or) structure of endocrine glands or endocrine tissues, including abnormal hormone source, abnormal hormone receptor, and physiological disorders caused by abnormal metabolism of hormones or substances.

　　The effects of estrogen on male feminization of female fetuses vary at different stages of pregnancy. If sex hormones are taken before 12 weeks of gestation, the closure of the lip pouch is more obvious; if sex hormones are used after 12 weeks of gestation, the clitoris enlargement is more obvious, and sometimes the vaginal opening is at the urethra, but it is rare. The degree of feminization in female fetuses after taking androgens seems to be lighter, and the enlarged clitoris can gradually shrink after birth.

　　This disease is caused by abnormal sexual differentiation induced by increased endogenous androgens and exogenous androgens, including progesterone with potential androgenic activity used during the first trimester of pregnancy (19-nor testosterone derivatives, including norethindrone, norethisterone, ethynodiol, danazol, gestrinone, levonorgestrel, and medroxyprogesterone), and androgen preparations, leading to virilization. Overdosage of diethylstilbestrol can lead to male feminization, so these drugs should be avoided before or during pregnancy.

　　Non-adrenal medullary hyperplastic pseudohermaphroditism patients should undergo sex chromosome testing, most of which are positive. If this test does not conform to normal males, chromosomal karyotyping should be performed, and the karyotype analysis of tissue cells is more helpful in identifying mosaics. For those with karyotype XX, careful search for female masculinization phenotypes should be made, and various adrenal hormones, 17-ketosteroids, pregnenolone, and 17-dehydroprogesterone should be measured to exclude common types of congenital adrenal hyperplasia. The diagnosis can be confirmed if ovarian and testicular tissue are found in histological examination. However, sometimes diagnosis is difficult due to abnormal development of the gonads. The sex chromosome is positive, and the karyotype is 46XX. 24-hour urine 17-ketosteroids and pregnenolone levels are elevated. Ultrasound and CT examinations sometimes show bilateral adrenal enlargement or lesions.

　　Postoperative dietary recipe for non-adrenaline hyperplastic pseudohermaphroditism:

　　1. How to make stewed turtle:750 grams of turtle, 100 grams of ham, 13 grams of starch (broad bean), and 25 grams of fresh mushrooms. Ingredients: 15 grams of yellow wine, 60 grams of scallion, 1 gram of monosodium glutamate, 100 grams of garlic, 3 grams of salt, 100 grams of refined lard, 25 grams of vinegar, and 1 gram of white pepper. Kill the live turtle, remove the internal organs, blanch, remove the black membrane, cut into pieces, rinse with water and set aside. Clean the ginger and cut into pieces, peel the garlic and crush into a paste. Cut the bamboo shoots into slices. Cut the ham into slices. Put the turtle pieces into a pot, add scallion whites, ginger pieces, cooking wine, bamboo shoot slices, and ham slices, add enough water to cover the turtle, bring to a boil on high heat, then simmer over low heat for 2 hours, remove the ginger and scallion, add salt and monosodium glutamate for seasoning, sprinkle with garlic paste before serving.

　　2. Silver ear pigeon soup:500 grams of squab, 10 grams of dried silver ear, 25 grams of scallion, 4 grams of ginger, 8 grams of salt, and 10 grams of yellow wine. Clean and dry the squab, cut off the head and feet; soak the silver ear in hot water, change the hot water every 10 minutes, repeat three times, cut off the base and yellow spots, clean and dry, and scatter into small pieces; place the soup pot on the fire, pour in chicken soup, add the squab and ginger slices, bring to a boil, add cooking wine, simmer over low heat for about 1 hour; add the well-soaked silver ear, and simmer until the soup is thick and the meat is tender; take out the squab, remove the bones, tear the pigeon meat into strips; put it back into the boiling pot, sprinkle with scallion, season to taste and it is ready.

　　3. Yam and pork ribs stew:250 grams of yam, 150 grams of pork ribs (large ribs), 5 grams of scallion, 3 grams of salt, 2 grams of ginger, 2 grams of vinegar, and 2 grams of star anise. Peel and clean the yam, cut into blocks; cut the pork ribs into pieces; put the yam and pork ribs into the inner pot of the pressure cooker, add scallion, ginger, refined salt, vinegar, star anise, and enough water, adjust the pressure to the pork ribs setting, and keep the pressure for 10 minutes, then it can be eaten.

　　4. Tangerine peel and Wuguyu chicken soup:400 grams of Wuguyu chicken. 10 grams of dried tangerine peel, 15 grams of white atractylodes, 15 grams of Poria cocos, 5 grams of purple carriaconcha, 15 grams of yam, 10 grams of ginger, and 3 grams of salt. Clean the Wuguyu chicken; peel and clean the yam, cut into irregular blocks; peel and clean the ginger, slice it; put water in the pot, bring it to a boil on high heat, add the Wuguyu chicken, skim the foam after boiling; add dried tangerine peel, white atractylodes, Poria cocos, yam slices, ginger slices, and refined salt, turn to medium heat, and boil for about 60 minutes; when the Wuguyu chicken is tender, pour out the soup, add the powder of purple carriaconcha, stir well, and drink it.

　　5, Astragalus and Codonopsis Stewed Old Chicken:600 grams of hen, 20 grams of Angelica sinensis, 20 grams of Astragalus membranaceus, 10 grams of dried dates, 4 grams of salt, 1 gram of monosodium glutamate. Cut the old hen into pieces, rinse it with water to clean the blood; wash Angelica sinensis, Astragalus membranaceus, and dried dates with water; bring water to a boil in the pot, blanch the cleaned chicken pieces; put the blanched chicken pieces, Angelica sinensis, Astragalus membranaceus, and dried dates into a炖盅, add water, place it on the fire and bring it to a boil, skim the foam on the surface; cover the lid, turn the heat to low, and continue to simmer for about 3 hours; when the chicken pieces are tender, add salt and monosodium glutamate to adjust the taste.

　　6, Stir-fried Blood Duck:Duck 2000 grams, black pepper 1 gram, ginger 6 grams, salt 4 grams, chili (red, pointed, dried) 5 grams, monosodium glutamate 3 grams, scallion 15 grams, garlic (white skin) 25 grams, peanut oil 30 grams, sesame oil 10 grams, cooking wine 35 grams, soy sauce 4 grams. Take a clean bowl, first add 15 grams of cooking wine, cut a knife from the neck of the duck, let the duck blood flow into the bowl, and stir with chopsticks; then immerse the duck in boiling water for a brief scald, then pluck the feathers, open the abdomen, remove the internal organs, cut into 1.8 cm square pieces, and put them in another bowl for later use (head, feet, wings, internal organs, etc. are not used); wash the ginger clean, cut into 1.2 cm square thin slices; remove the roots of the scallion, wash it clean, and cut the white part into 1.2 cm long segments; slice the dried red chili diagonally into 0.9 cm long strips; cut the garlic in half and put it all in a clean bowl; heat the iron pot over high heat, pour in peanut oil, and heat it to 70% heat, then add the chopped ginger, scallion, garlic, and dried red chili, and stir-fry until fragrant; then add the duck pieces and stir-fry together; when the duck pieces shrink and turn white, add 20 grams of cooking wine, soy sauce, and refined salt and stir-fry again; then add 200 milliliters of fresh soup, move the iron pot to a low heat and simmer for 10 minutes; when the soup is about 1/10 remaining, pour the duck blood over the duck pieces, stir while pouring, so that the duck pieces are covered with duck blood, and pour it after adding black pepper, monosodium glutamate, and stir-fry slightly, then turn off the heat, put it in a plate, and pour sesame oil on top.

　　The masculinization of non-adrenal hyperplastic female pseudohermaphroditism is not severe, so the treatment is relatively simple and easy to see results. If necessary, the clitoris or the part of the labial sac that is closed should be excised, the urethral orifice and vagina should be exposed, and slight repair should be made. For this type of female masculinization, female upbringing should be practiced.