XY dysgenesis gonadal: āhuatanga o te kōwhiringa mate me te maimoatanga

XY dysgenesis gonadal - te reira rawa he mate congenital onge, te whanaketanga o e te tikanga he takahi o te hanganga Y-pūira (e korero tatou e pā ana ki te ngaro o te ira motuhake irakētanga o wehe ranei).

Ko te take o te mate

Tikanga, te take tonu o tenei mate ko te irakētanga ira o te tetahi ira, kei roto i te ringa poto o te Y-pūira, mate katoa o te ira ranei. Tenei pūira te kawenga mō te kōtuitanga pūmua, tango wahi i roto i te takoto o te whanaketanga kukune o momo tane. Ka rite ki te hua, no te mea te pānga o te taiaki tane he ngaro, te kukune, ko te kotahi anake kōwhiringa - ki te hanga i te momo wahine. Ka rite ki te hua, kua ki te tamaiti e parauhia te tohuāhua wahine ki karyotype "XY".

pathogenesis

Irakētanga ngaro o SRY ira ranei arata'i ki pārōnaki hē Sertoli pūtau, ka rite te tupu, ki te maldevelopment o nga tubules seminiferous.

Ka rite ki te hua, i roto i te noa'tu o te "tautāne" te karyotype XY, kua hanga e ka whakatakotoria i runga i te momo wahine whekau fakasekisualé o te kukune.

faaiteraa haumanu

Tae noa te timatanga o tohu pūhuruhurutanga titiro tata Svayera syndrome kore whakahuatia. Ko reira anake rite tupu ratou timata kōtiro pakeke ki te whakaatu i etahi āhuatanga:

• Te kore o te huruhuru te tinana, me te wāhi tuku whakararo o te taihemahema waho.
• Takarepa, whanaketanga rawakore o te repe mammary.
• Taumata o hypoplasia, te infantilism kōpū.
• hypoplasia tara (iti noa).
• Ata korerotia āhuatanga fakasekisualé tuarua - "evnuhopodobny" ranei momo tinana intersexual.
• Hypotrophy atrophy o te mucosa o nga kikokiko ranei.
• Hypoplasia o te taihemahema waho (labia me tonetone).
• infantilism taihemahema.
• I roto i ētahi wā i reira he tupu nui o te tinana kaha, me ona wahi: te kauae raro, ko te whitiki pokohiwi (hua i roto i te hanganga pokohiwi whanui) uaua.
• Ko te tīmatanga o te pūhuruhurutanga i roto i ngā kōtiro ki te Svayera syndrome taea e tika ki te kore o te estrogen i roto i o ratou tinana.
• Whakaoti sterility.

tātaringa

I roto i te nuinga o te wā pāngia te mate te i te tau o 15-16 tau, i roto i te pūhuruhurutanga o, ka riro te reira mārama e kore i te manawanui korerotia tuarua āhuatanga fakasekisualé.

I te wa ano, kei te tīmata wahine e whai i te irakētanga, pa tonu tenei tau ki te tahuri ki te rata mātai ki amuamu o roa i roto i te tīmatanga o te paheketanga.

I te tahi taime kawea te tātaritanga i roto i rite ki te hua o te dysplasia me repara gonads underdeveloped.

hāngai ana te tātaritanga o "XY dysgenesis gonadal" kei runga i te āhuatanga e whai ake nei:

• whakamātautau tinana o te manawanui.
• Ultrasound whakamātautau.
• Hysterosalpingography.

Heoi, he taea haamauraa o te tātaritanga anake i te tikanga o te ako o te sex pūpūira, e kitea te aroaro o te karyotype tane ki te tohuāhua wahine.

Kōwhiringa maimoatanga

Treatment o te syndrome kawea Svayera i roto i te maha ngā tohutohu.

1. Matua hua spaying - e tika ana ki te tūponotanga nui o ratou faafariuraa ki te pukupuku mārere.
2. I muri i ovariectomy whakaritea maimoatanga substitutive ki āpiha hormonal. whai wāhi ana tēnei ki te whanaketanga o ngā āhuatanga fakasekisualé tuarua.
3. A, no te te pai whakawhanakehia te kōpū, i reira te mea he pea o te hapūtanga me te tamaiti hauora (puta hapūtanga rite ki te hua o roto i vitro te kikiritanga).

Me kia taiea i te syndrome rite i te ingoa Svayera-James-MacLeod te mate. Tenei huru, me te rite i roto i faaiteraa lymphangioleiomyomatosis, he māuiuitanga e pa i te kiko tana pukapuka. XY dysgenesis gonadal me lymphangioleiomyomatosis - rerekē mate.