Albright syndrome. Syndrome Mc Kyung-Albright-Braitseva. Take, maimoatanga

ko Syndrome Mc Kyung-Albright i roto i te honore o te rua kaiwhakaako tairanga, e whakaahuatia ana e ia, nui atu i te hawhe te rau tau ki muri. ka korerotia e ratou i te iwi whānui e pā ana ki te tamariki, e te nuinga o nga kotiro. He tokomaha o ratou i poto roa, mata a tawhio, kaki poto, IV poto me te V metatarsal ranei wheua metacarpal i mamae uaua, huringa i roto i te kōhiwi, te hekenga teething tohu hypoplasia. Na tera ano hoki he hinengaro retardation me endocrine mate, faaite pūhuruhurutanga wawe ki toto paheke, te whanaketanga o u, tupu o te makawe tore me u, te hua i roto i te tere o te tupu o ngā tamariki, me ngā rerekētanga i roto i te kiri.

I roto i te rongoā hou, te wā "syndrome Albright" Kei te whakamahia ki tūroro e whai katoa anake etahi endocrine me te kiri abnormalities ranei. He take i reira te tātaritanga whakaturia i roto i te kōhungahunga. Heoi, i roto i ngā take angamaheni, kua hoatu ai ki ngā tamariki 5-10 nga tau, e hāngai ana i runga i nga tohu, i he āhuatanga o tenei mate. Ko te tikanga ko reira he onge, me te riro. tonu te reira unknown rite te etiology me pathogenesis o tenei mate. Kia titiro a ki nga tohu o te mate.

Maumaui i roto i te pūnaha endocrine

More maha atu i te mau tamahine, ki kitea Albright syndrome te precocious pūhuruhurutanga, meinga nei e estrogens tukua ki te toto i cysts ovarian. kia ake i cysts i muri i te iti iho i roto i te rahi i roto i te wiki torutoru ra ranei. Ki te awhina o ngā tukanga ultrasound i te whai wāhi ki te kite me te ki te ine i te rahi o te pukupuku. Ka taea e cysts tupu ki rawa he rahi tika. He nga take, ka i tupu ai ki te rahi o te korowha pōro, e te mea neke atu i te 50 mm te whānui.

E kitea uma augmentation me toto paheketanga me te tupu o cysts. Ki te timata te kotiro paheke ana i mua i tau 2, ko reira te tohu tuatahi o Albright syndrome. Heoi, e taea te kite i te aroaro o te paheketanga me te ovarian cysts kōhikohiko e rua i roto i taiohi me nga wahine pakeke. Tenei e kore katoa aukati i ki te whai tamariki hauora.

Maimoatanga o ngā tamariki ki te pūhuruhurutanga precocious, rawa uaua, me te hauarea. Ahakoa, ki te hāparapara nekehia te whēwhē, i taea anō-puta i te reira. A, no te tango i te progesterone taiki e taea te mutu paheke ana, engari e kore e te te auau tere o te whanaketanga wheua, me te tupu tērā iho. pānga kino pea mo te mahi o te repe adrenal. I roto i te maimoatanga o formulations whakamahia mō te ingestion ārai estrogen kōtuitanga.

mahi thyroid

50 ōrau o te iwi ki Albright syndrome, e mate ana i disorders o te repe thyroid. Tenei-ka karanga kia goiter, nodules me cysts. He pea take onge mohio huringa hanganga. I roto i enei tūroro, he iti te taumata whakaputaina e te TSH rapae o, engari nga taumata o hormones thyroid he uara noa paku faateiteihia ranei. Tukinotia, i te tikanga o e heke kōtuitanga o hormones thyroid. Kei te tohua te reira i roto i te wā i reira he nui nui ki te tuku i te taiaki te taumata.

secretion nui o taiki

A, no te tīmata te rapae o mate ki te huna nui o taiki tupu. I roto i te tamariki, kua oti nei te hoatu tenei tātaritanga rite Albright syndrome, i kitea acromegaly. timata nga tangata taitama ki te puta makaka āhuatanga kanohi, hohoro tupu ringa me waewae, i taea e mamae ratou rumati. iti maimoatanga o ngā tamariki ki taua tohu e ki te tango hāparapara o te rohe rapae o me te whakamahi o analogs somatostatin taiaki waihanga, inhibits te hanga o te taiki.

Ētahi atu disorders endocrine

He secretion onge, me te nui o te whakarahi adrenal. kia hua te pakaru taua i roto i te mōmona te tinana me te mata, he hua i roto i te taimaha tinana, paipa o tupu me te fragility kiri. enei tohu katoa i huaina syndrome o Cushing. A, no te e nekehia atu taua huringa pāngia rongoā adrenal tango ranei e whakaiti i te kōtuitui o cortisol.

I te tahi taime i tamariki Albright syndrome, kitea i roto i te toto taumata tino iti o te ūkuikui no o te mate nui o te Phosphate i te mimi. Ka taea e tenei mate e te take o huringa wheua e pā ana ki kōiwi. Ka rite ki te maimoatanga me te Phosphate-waha, me te huaora D i roto i te tua.

Disorders e pā ana ki te kiri

I runga i te kiri i te whanautanga tata muri puta wahi o te tae "kawhe ki te waiu" ranei. Ratou tino maha puta i runga i te sacrum, kātua, waewae, papa, hoki o te kaki, rae, tumuaki, hoki o te matenga. E hoki katoa o ratou he tohu e te tamaiti Albright syndrome. Ka taea te kite i Photos o enei wahi i raro.

Ahakoa tenei mate ko neurofibromatosis, ano hoki i tae wahi "kawhe ki te waiu". Heoi, te āhuatanga Albright syndrome e wahi nui ki fokotu'utu'u kōhikohiko, he iti i roto i te maha ratou. E ratou he diameter o i te 1 ki te henimita torutoru, kauruku parauri. Tae te taua katoa, he porotītaha i āhua ratou, kua āhuatanga ratou e te mata maheni. I te whakamātautau histological maha heheu e kore te te kiriwaho ke i roto i tona hanganga, engari ka nui haere te nui o te melanin i roto i nga keratinocytes paku.

wahi taratahi o tenei momo e taea hoki e kitea i roto i te iwi te kore hauora. Ki te kore ratou e whakararuraru i a kahore e tupu, na e kore te mea e tika ana te maimoatanga. Ki te reira he tupu tere, i reira e kōhikohikotia wahi ahuahu, e tūtohu ana ki te tūhura i ratou histologically. Na ka nekehia atu hāparapara.

mutunga

Ko te kupu, e nehenehe tatou e te mea e hoki Albright syndrome āhuatanga e mate o te wheua ranei angaanga, te aroaro o wahi ngarahu kiri, pūhuruhurutanga wawe. Ahakoa i reira he take te wahi i reira e anake nga tohu tuatahi e rua. I roto i te whānui, i te āhuatanga matua o te syndrome ko pāpaka wheua (osteodysplasia). Heoi, mutu tenei tukanga ina pūhuruhurutanga. I roto i te pakeke, e kore e ahu whakamua huringa wheua. Ko te tikanga, ko te kite, me te maimoatanga tika, ko tino pai te waitohunga o te therapy o tenei mate.