Androgenic unresponsiveness
| Androgenic unresponsiveness | |
|---|---|
 AIS results when the function of the androgen receptor (AR) is impaired. When I have functional decline of The AR protein (pictured) mediates the effects of androgens in the human body. → androgen receptor (AR), it develops. The AR protein (figure) brings the human body an androgenic effect. | |
| A classification and outside reference information | |
| Clinical department ・ Academic field | Endocrinology |
| ICD-10 | E34.5 |
| ICD-9-CM | 259.5 |
| OMIM | 312300 300068 |
| DiseasesDB | 29662 12975 |
| MedlinePlus | 001180 |
| eMedicine | ped/2222 |
| MeSH | D013734 |
| GeneReviews | |
Androgenic unresponsiveness (androgenic ふおうしょう) (British: Androgen Insensitivity Syndrome, abbreviated to AIS) is one of the diseases causing the nature differentiation disease.
Table of contents
Summary
I am classified in male pseudohermaphroditism. An obstacle occurs for the sex differentiation to a man without being able to sense androgenic all or part because an androgen receptor does not act although I can secrete a male hormone (androgen). It is said with part type androgen unresponsiveness (PAIS) with the thing which does not sense archetype androgen unresponsiveness (CAIS), a part with the thing which does not sense androgenic all.
Androgenic unresponsiveness patients usually have XY types (male model) as sex chromosomes (even if it is androgenic unresponsiveness if it is a woman model of XX, there is not the symptom in particular, and they are not discovered as a disease, but it is carrier ). Therefore the gonad has a testis, but stays in the body (stoppage testis). Because the external genitalia does not react to androgen, I become nature to a woman model in the archetype, and it is often in the form that is hard to distinguish it with the woman type with the man type with the partial type.
It is rare that it is found out at the time of birth in the archetype and is brought up as a normal girl. The gender identity is a woman, too. Because the menarche does not come even if it becomes pubertal, and secondary sex character happens (primary amenorrhea), I receive the obstetrics and gynecology department, and the cases that this disease is discovered are often found. Apparently the vagina lasts in normal women, but it ends in blindness edge (I do not beat) (an edge is occupied) and may not have the length that is necessary for sex. Because I do not have the uterus, the pregnancy, the delivery is impossible. I may be brought up as a boy with the partial model by a shape of the external genitalia.
The androgenic unresponsiveness is X chromosome-dependent sex-linked inheritance, and mother is often a carrier.
Correspondence
In fact, having been "which is 46,XY where a man has many chromosomes" (relatively rare for a woman), the pregnancy, fear to give the impressionable pubertal woman including the delivery being impossible a big mental blow are big diseases. Therefore mental care becomes most important. Even if androgenic unresponsiveness is diagnosed, in the archetype in particular, people living as the same woman are often found.
In addition, it is said that it is necessary to extract it early because the stoppage testis is at increased risk for cancerating, and to be operated on, but testis extraction is performed the puberty when secondary sex character completed it now later because it rarely cancerates before an adult. This is because quantity of hormone necessary for secondary sex character is independent, and it is short prepubertally when it removes a gonad. In addition, even general man and woman is so, but a part of the secreted male hormone is converted into female sex hormone in the body and functions, and it is an important source of supply for the hormone secretion from a testis to form growth of the breast and a feminine figure even if it is the androgenic unresponsiveness of the archetype. I supplement female sex hormone by hormone replacement therapy to prevent menopausal disorders after testicular extraction. The hormone replacement therapy must be cautious about the judgment of the testicular extraction to last for life. I receive examination diligently and may follow it up without extracting a testis. I am seen with (2%) that is not high so that the part-shaped stoppage testis does extraction with requisiteness for one (50%) of that a canceration risk is high in the archetype in particular daringly. [1]
I go 造膣術 () with the case which vagina has a short and may extend vagina.
Alias
The androgenic unresponsiveness is called testicular (the nature) feminization symptom (候群), testicles (the nature) feminization symptom (候群) (Testicular feminization syndrome). Because it is told a patient to have a big mental blow, and this disease name had the request of the patient person concerned, I am hardly used now.
Allied book
- Blue bow company, 2004 when "but even a man has a woman, and Hideo Hashimoto lives in sex full version - intersex" (hermaphroditism). ISBN 4-7872-3227-4。 OCLC 123057441.
- It is 565-578 pages, ISSN 00016543, NAID 10024132484 "an agreement opinion (PDF) about the management of the abnormality symptom nature" Tsutomu Ogata, Reiko Horikawa, 長谷川奉延, others on "Japan pediatrics society magazine" Vol. 112 third, Japanese pediatrics society, March 1, 2008.
Allied item
- Sex differentiation disease
- Intersex
- Hermaphroditism
- A jazz singer with this Eden Atwood (Eden Atwood) - disease.
Outside link
- The Secret of Women with Male DNA (English)
- AIS/DSD Support Group for Women and Families (the support group for women of the androgenic unresponsiveness: the United States) (English)
- Androgen Insensitivity Syndrome Support Group (an androgenic unresponsiveness support group: the U.K.) (English)
- Information site NCXX DSD Japan (Japan) of a child and the family having DSD (sex differentiation disease)
- A sex differentiation disease: Handbook (Japan) for families
- Support movie (Japan) of people and the family with a development state of the nature of various bodies (sex differentiation disease)
Footnote
This article is taken from the Japanese Wikipedia Androgenic unresponsiveness
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