Albright syndrome. Syndrome Mc Kyung-Albright-Braitseva. Zvinokonzera, mabatiro

Syndrome Mc Kyung-Albright akanzi kuremekedza vanachiremba vaviri yokufeva, uyo akarondedzera kupfuura hafu adarika. Vakaudza voruzhinji nezvevana, vazhinji vacho vasikana. Vakawanda vavo vaiva nomumhu, kumativi chiso, mutsipa pfupi, pfupi IV uye V metatarsal kana mapfupa metacarpal akanga kwetsandanyama, zvachinja marangwanda, kunonoka teething enamel hypoplasia. uyewo paiva mupfungwa retardation uye zvirwere kwemakemikari anobudiswa, akaratidza mangwanani kuyaruka vane kumwedzi ropa, kugadzira mazamu, ikure pubic bvudzi uye mazamu, kuwedzera mwero kukura vana uye kuchinja paganda.

In mushonga ano, mashoko okuti "Albright chirwere" rinoshandiswa varwere kuti vane zvose kana chete vamwe kwemakemikari anobudiswa uye ganda abnormalities. Pane zviitiko apo nechirwere panzvimbo muduku. Zvisinei, muna mumufananidzo kacho anoiswa vana makore 5-10 okuberekwa, inobva zviratidzo, izvo runozivisa chirwere ichi. Kazhinji ndiwo isingawanzowanikwi uye nhaka. Zvazvinosarira asingazivikanwi sezvo etiology uye pathogenesis chirwere ichi. Ngatimboonai nezviratidzo chirwere.

Kutyorwa ari kwemakemikari anobudiswa mugadziriro

Kakawanda kupfuura vasikana, pamwe Albright chirwere ari kuchengetwa precocious kuyaruka, izvo zviri kukonzerwa estrogens akabudiswa mugomba ropa kubva ovarian cysts. Cysts kungawedzera pashure kuderera kukura mukati shomanana kana mazuva. Achibatsirwa Ultrasound kurapa vane mukana kuona uye kuyera kukura anomera. Cysts vanogona kukura kuva zvikuru yekutenga saizi. Paiva nguva apo yakanga yakura kukura gorofu bhora, kuti inopfuura 50 mm mu dhayamita.

Zamu augmentation uye kumwedzi ropa vari kuchengetwa pamwe kukura cysts. Kana musikana akatanga kumwedzi pamberi zera 2, ndicho chiratidzo Albright urwere rokutanga. Zvisinei, kuvapo kwemashifiti kumwedzi uye ovarian cysts zvinogona kuonekwa zvose kuyaruka uye akura vakadzi. Izvi zvose hakutadzisi kuva nevana hwakanaka.

Treatment vana vane precocious kuyaruka, zvakaoma uye va neudhadha. Kunyange kana chikofu surgically abvisa, zvinogona kuzodzosera kubuda. Kana kutora kemikari progesterone inogona vakarega kumwedzi, asi nhamba nokukurumidza pfupa kukura uye kukura harisi kuderera. Zvingangoitwa chokuita basa adrenal mumasero. In pakurapa vakashandisa Formulations kuti ingestion ichivharira inonzi estrogen usage.

thyroid basa

50 muzana yevanhu vane Albright urwere, kutambura kubva zvirwere zviri thyroid shwanu. Izvi zvinonzi goiter, nodules uye cysts. Pangava zvisingawanzoitiki zviitiko dzisinganyatsooneki STRUCTURAL kuchinja. In varwere izvi, padanho zvinoberekwa pituitary TSH yakaderera, asi siyana pamusoro thyroid mahormone hwemazuva tsika kana zvishoma chakakwirira. Zvakaipa, hwaishandiswa zvinoderedza kwetunhu thyroid mahormone. It unoratidzwa zviitiko apo padanho iri kumusoro zvakakwana kusunungura kemikari.

Kunwisa secretion kukura kemikari

Kana pituitary chirwere anotanga secrete yakawanda kukura kemikari. In vana, rave kuisa chirwere ichi sezvo Albright urwere, acromegaly yakawanikwa. Majaya vakatanga kuonekwa zvinonyadzisa chiso features, akakurumidza akakura maoko nemakumbo, vaigona kutambura chearthritis. Treatment vana vane zviratidzo zvakadai ipfupi kuti kuvhiya kubviswa pituitary nharaunda uye kushandiswa chakaita zvokugadzirwa kemikari somatostatin analogs, kuchititadzisa kugadzirwa kukura kemikari.

Other kwemakemikari anobudiswa zvirwere

Kune mashoma uye kunyanya secretion pamusoro adrenal nedumbu. Kwakadaro rwakavhunika angaguma kufutisa mumuviri uye chiso, kuwedzera uremu muviri, kumira kukura uye ganda fragility. Zviratidzo zvose izvi rinonzi Cushing kuti syndrome. Kana kuchinja kwakadaro vanobviswa sei adrenal kana kutora mishonga kuti kuderedza kwetunhu Cortisol.

Dzimwe nguva vana vane Albright urwere, akacherechedza muropa yakaderera chaizvo kwevari phosphorus nokuda guru kurasikirwa phosphate iri weti. chirwere ichi chinogona kuva chikonzero pfupa kuchinja chokuita kusimba. Sezvo kurapwa pamwe nomumukanwa phosphate uye vhitamini D Kuwedzera.

Zvirwere chokuita ganda

Paganda pakuzvarwa kana nguva pfupi pashure pacho kuzviratidza makwapa ruvara "kofi mukaka". Ivo kazhinji dzinoitika pamusoro sacrum, hunde, makumbo, magaro, makotsi, pahuma, romusoro, shure musoro. Vose zvavo vari zvakare chiratidzo chokuti mwana Albright syndrome. Photos yenzvimbo idzi dzinogona kuonekwa pazasi.

Kunyange zvazvo chirwere ichi ndicho neurofibromatosis, vanewo gwapa ruvara "kofi mukaka". Zvisinei, Albright chirwere kunoratidzwa makuru makwapa paunowana hurukuro, vari vashoma mune nhamba. Vane dhayamita inobva 1 mishoma masendimita, mashava tint. Ruvara zvose zvakafanana, vari denderedzwa iri chimiro, ivo dzinoratidzwa kwakanaka penyika. On histological kuongororwa kazhinji hunoratidza kuti Epiderme e hakuna kushanduka kwaro mamiriro, asi uwandu rwe-melanin ari keratinocytes yakawedzera zvishoma.

Isolated makwapa izvi mhando kunogona kuwanikwa vanhu zvimwe hwakanaka. Kana havatsvaki uye regai kukura, zvino kurapa hakusi zvakakodzera. Kana paine Kuwedzera pane irregularly shaped makwapa, Zvinokurudzirwa kuti kuongorora kwavo histologically. Zvino akabvisa surgically.

mhedziso

Saka, tinogona kutaura kuti Albright chirwere kunoratidzwa kurasikirwa pfupa kana dehenya, kuvapo ganda ruvara makwapa, mangwanani kuyaruka. Kunyange kune zviitiko apo pane chete zviratidzo zviviri chokutanga. Kazhinji, chikuru chepfungwa chirwere ndiyo mapfupa maronda (osteodysplasia). Zvisinei, izvi rinomira kana kuyaruka. In vakuru, mapfupa kuchinja havasi kufambira mberi. Kazhinji, kuonekwa uye kurapwa, asi fungidziro yokurapa zvirwere izvi zvikuru zvakanaka.