Albright syndrome. Syndrome Mc Kyung-Albright-Braitseva. Zoyambitsa, chithandizo

Syndrome Mc Kyung-Albright dzina lake polemekeza madokotala awiri otchuka, amene analongosola oposa theka zapitazo. Iwo anauza anthu za ana ambiri amene anali atsikana. Ambiri mwa iwo anali aafupi, kuzungulira nkhope, lalifupi khosi, lalifupi IV ndi V metatarsal kapena mafupa metacarpal anali spasms minofu, kusintha mu mafupa a, anachedwa teething enamel hypoplasia. Palinso anali retardation ndi maganizo matenda endocrine, anasonyeza msinkhu mwamsanga magazi kusamba, kukula kwa mawere, kukula kwa tsitsi obisika ndi mawere, kuchuluka kwa mlingo wa kukula kwa ana ndi kusintha kwa khungu.

Mankhwala ano, mawu akuti "Albright syndrome" ntchito odwala kuti onse kapena ena okha endocrine ndi khungu kuvutika. Pali nthawi imene matenda anapereka ana aang'ono. Komabe, m'zochitika lililonse, ndi kuyika kwa ana zaka 5-10, zochokera zizindikiro, zimene khalidwe la matenda. Nthawi zambiri ndi osowa ndi akatengela apabanja. Patsalanso sakudziwika ngati etiology ndi pathogenesis za matenda. Tiyeni tione zizindikiro za matenda.

Kuphwanya mu dongosolo endocrine

zambiri kuposa atsikana, ndi Albright syndrome zimawonedwa precocious msinkhu, amene amayamba ndi estrogens anamasulidwa mu magazi kuchokera cysts yamchiberekero. Cysts achuluke pambuyo kuchepa kukula mwa masabata angapo kapena masiku. Mothandizidwa ndi njira ultrasound ndi mwayi kuti awone ndi kuyeza kukula kwa zotupa. Cysts akhoza kukula ndithu wamakhalidwe. Panali milandu, pamene nkulu kukula kwa gofu mpira, uposa 50 mm m'mimba mwake.

M'mawere augmentation ndi magazi kusamba ali anaona pamodzi ndi kukula kwa cysts. Ngati mtsikana anayamba kusamba asanafike zaka 2, ndi chizindikiro choyamba cha matenda Albright. Komabe, pamaso pa msambo ndi yamchiberekero cysts osasamba zingaoneke onse achinyamata wamkulu akazi. Izi zonse samaletsa ndi ana athanzi.

Chithandizo cha ana ndi msinkhu precocious, kovuta ndi inefficient. Ngakhale chotupa opaleshoni yochotsa, izo zikanakhoza kachiwiri kutuluka. Pamene akutenga timadzi progesterone akhoza anasiya kusamba, koma kugunda kwambiri chitukuko fupa ndi kukula si liwiro. N'zotheka zotsatira zoipa ntchito ya zopangitsa kwa adrenal. Mankhwalawa formulations ntchito ingestion kutsekereza esitirojeni kaphatikizidwe.

chithokomiro ntchito

50 peresenti ya anthu ndi matenda Albright, akudwala matenda a chithokomiro England. Izi otchedwa Chotupa chija, tinatake tozungulira ndipo cysts. Pangakhale nthawi zina wochenjera kusintha structural. Odwala awa, mlingo opangidwa ndi pituitary TSH ndi otsika, koma amaudindo mahomoni chithokomiro mfundozi yachibadwa kapena pang'ono okwera. Ankachitira, njira imene amachepetsa kaphatikizidwe mahomoni chithokomiro. Iwo anasonyeza kuti tidziteteze msinkhu ndi mkulu mokwanira kuti amasule timadzi.

Kugwiritsa katulutsidwe kukula timadzi

Pamene matenda pituitary akuyamba secrete yambiri kukula timadzi. Ana, amene achita matenda ichi matenda Albright, acromegaly anatulukira. Anyamata anayamba kuoneka mbali coarse nkhope, mofulumira kwambiri manja ndi miyendo, iwo akhoza kuvutika ndi nyamakazi. Chithandizo cha ana ndi zizindikiro chotero yafupika kuchotsa opaleshoni ya m'dera pituitary ndi ntchito kupanga timadzi somatostatin analogs, kumabwezera mmbuyo kupanga kukula timadzi.

matenda ena endocrine

Pali kawirikawiri ndi kumwetsa katulutsidwe wa kukulitsa adrenal. Chotero zolakwikazo ndi angachotsedwe kunenepa thupi ndi nkhope, akuchuluka mu thupi, kutha kwa kukula ndi khungu fragility. Zizindikiro zonsezi amatchedwa matenda Cushing a. Kusintha amenewa achotsedwa anakhudzidwa mankhwala adrenal kapena kutenga kuti kuchepetsa kaphatikizidwe tochepa.

Nthawi zina ana Albright syndrome anati m'magazi pamlingo wochepa kwambiri wa phosphorous chifukwa cha imfa waukulu wa mankwala mu mkodzo. matendawa akhoza kukhala chifukwa cha kusintha fupa kugwirizana ndi rickets. Monga mankhwala ndi mankwala m'kamwa ndi vitamini D kuwonjezera.

Matenda kugwirizana ndi khungu

Pa khungu pobadwa kapena pasanapite nthawi kuoneka mawanga a mtundu "khofi ndi mkaka". Iwo nthawi zambiri zimachitika pa sacrum, thunthu, miyendo, matako, kumbuyo kwa khosi pamphumi, khungu, kumbuyo kwa mutu. Onse a iwo ali chizindikiro kuti mwana Albright syndrome. Photos mawanga izi Tingaone pansipa.

Ngakhale matenda ndi neurofibromatosis, komanso ndi malo mtundu "khofi ndi mkaka". Komabe, syndrome Albright amakhala ndi mawanga zazikulu ndi zazikulu osasamba, iwo ali ochepa mu chiwerengero. Iwo ndi awiri a 1 ndi masentimita angapo, zofiirira kulocha. Mtundu lomwelo, iwo ali chowulungika mu mawonekedwe, iwo yodziwika ndi yosalala pamwamba. Kuzionetsetsa histological zambiri kwavumbulutsa kuti khungu ndi sanasinthe mu dongosolo lake, koma kuchuluka kwa melanin mu keratinocytes chinawonjezeka pang'ono.

mawanga akutali a mtundu akhoza kupezeka anthu mwinamwake wathanzi. Ngati iwo musavutike ndipo musati kukula, ndiye mankhwala Sikuti. Ngati pali kukula mofulumira, pali zoumbika mosiyanasiyana mawanga, izo tikulimbikitsidwa kufufuza iwo histologically. Ndiyeno kuchotsedwa opaleshoni.

Pomaliza

Choncho, tingathe kunena kuti syndrome Albright amakhala ndi imfa ya fupa kapena Chigaza, pamaso pa mawanga khungu pigment, oyambirira msinkhu. Ngakhale pali milandu pamene pali zizindikiro awiri oyamba okha. Ambiri, mbali yaikulu ya syndrome ndi fupa zotupa (osteodysplasia). Komabe, njira imeneyi mabasi pamene akutha msinkhu. Akuluakulu, kusintha fupa si patsogolo. Nthawi zambiri, kudziwika ndi mankhwalawa kuti madokotala ananena zawo mankhwala a matenda ndi yabwino ndithu.