Albright syndrome. Syndrome Mc Kyung-Albright-Braitseva. Kubanga, unyango

Syndrome Mc Kyung-Albright igama imbeko oogqirha ababini amanqugwala, ngubani wazichaza ukuba ngaphezu kwesiqingatha kwekhulu eyadlulayo. Bathi uluntu malunga abantwana, uninzi lwabo amantombazana. Abaninzi kubo omfutshane ngesithomo, ubuso ngeenxa zonke, intamo emfutshane, IV elifutshane V amadeo okanye amathambo metacarpal zaye aqaqambayo, utshintsho kuphahla, Ilibazisekile ezingenakunqandwa wayesebenzisa hypoplasia. Kananjalo ngengqondo zigule nezifo yonyango, wavakalisa ukufikisa kwakudala ukopha sexesheni, uphuhliso amabele, kukhule uboya zikawonke wonke kunye amabele, ukunyuka izinga lokukhula abantwana kunye notshintsho esikhumbeni.

Xa yesilungu, igama elithi "Albright syndrome" lisetyenziswa ukuba izigulane ukuba zonke okanye kuphela ezithile ezidalwe nesikhumba ezingaqhelekanga. Kukho iimeko apho uxilongo emiselwe yabantwana yasekuqaleni. Nangona kunjalo, kwiimeko ezithile, usibeka kubantwana kwiminyaka 5-10 ubudala, esekelwe imiqondiso, leyo uphawu kwesi sifo. Ngokubanzi kube yinto enqabileyo yaye ilifa. Kuhleli ayaziwa njengoko umyalezo kunye Pathogenesis kwesi sifo. Makhe sijonge iimpawu zesi sifo.

Ukunyhashwa kwinkqubo yonyango

Usoloko amantombazana, kunye Albright syndrome emsebenzini kom lokufikisa, nto leyo ibangwa estrogen angene igazi cysts ovarian. Cysts kungandisa emva ukuncipha ngaphakathi kwiiveki ezimbalwa okanye iintsuku. Ngoncedo kweenkqubo ultrasound banethuba kubona kwaye ukulinganisa ubungakanani yamathumba. Cysts nga akhule kakhulu ubungakanani endilisekileyo. Kwakukho iimeko, xa lakhula kubungakanani ibhola yegalufa, ukuba ngaphezu kwe-50 mm ubukhulu.

Breast esele kwegazi exesheni kuvandlakanywa kunye nokukhula cysts. Ukuba intombazana eqale phambi kweminyaka 2, oko uphawu lokuqala Albright syndrome. Nangona kunjalo, ubukho sexesheni kunye ovarian cysts ezitenxileyo ibonakala kokubini abafikisayo abadala abafazi. Oku konke akuthinteli ukuba abantwana esempilweni.

Unyango kwabantwana lokufikisa kom, zicwadi kunzima kakhulu kwaye azibi. Nokuba cyst yasuswa intsumpa, oko re-kuvela. Xa uthatha i dlala hormone singamiswa osexesheni, kodwa isantya ngokukhawuleza zophuhliso amathambo kunye ukukhula behlise phantsi. Iimpembelelo ezingalunganga ezinokwenzeka ukuba umsebenzi adrenal. Xa unyango nemixube asetyenziselwa yokufakwa ekuvimbeni estrogen yokuqaphela.

umsebenzi lengqula

50 ekhulwini abantu Albright syndrome, abaneengxaki ze ingaba lengqula. Oku njalo kuxokwa-goiter, maqhuqhuva kunye cysts. Kusenokubakho amatyala enqabileyo ngokungangqalanga utshintsho lobume. Kwezi izigulane, inqanaba eveliswe TSH yebhinqa iphantsi, kodwa amanqanaba ze hormone amaxabiso eqhelekileyo okanye ephakamileyo kancinci. Ezinyangiweyo, esebenzisa apho unciphisa Yindibanisela hormone. Oku kuboniswe kwimeko apho umgangatho ophezulu ngokwaneleyo ukuze ukukhulula hormone.

secretion ngokugqithisileyo hormone

Xa yebhinqa sifo uqala cell ukuba izixa ezikhulu hormone yokukhula. Kubantwana, nto leyo eye wabeka unesifo njengoko Albright syndrome, acromegaly uyaziwa. waqala abafana ukuba kuvela iimpawu zobuso erhabaxa, ngokukhawuleza wakhula iingalo kunye nemilenze, akaba ubunzima samathambo. Unyango lwabantwana kunye neempawu ezifana kuyehla nokususwa zoqhaqho kummandla yebhinqa kunye nokusetyenziswa hormone eyenziweyo analogs somatostatin, abangaphangeliyo imveliso hormone yokukhula.

Nezinye izifo yonyango

Kukho secretion enqabileyo ngokugqithiseleyo ukukhula adrenal. ulwaphulo olunjalo kungabangela Ukutyeba umzimba kunye nobuso, ukwanda ubunzima bomzimba, ukumisa ukukhula nesikhumba ubu. Zonke ezi mpawu ekuthiwa Cushing engqondweni. Xa kususwa ezinjalo utshintsho abachaphazelekayo amayeza adrenal okanye ukuthatha ezinciphisa Yindibanisela cortisol.

Ngamanye amaxesha abantwana Albright syndrome, waphawula egazini kumazinga asezantsi kakhulu phosphorus ngenxa yokufelwa elikhulu phosphate yokuchama. Le disorder kuba unobangela lotshintsho bone ezinxulumene yamathambo. Njengoko unyango kunye phosphate yomlomo vitamin D ngaphandle.

Ukuphazamiseka ezinxulumene ne nesikhumba

Kwi esikhumbeni ekuzalweni okanye kamsinya emva koko kuvela amabala yebala 'ikofu ubisi ". Bona kaninzi kwenzeka sacrum, isiqu, imilenze, ezinqeni, emva entanyeni, ebunzi, Nentloko enesihlwitha yohambayo, emva entloko. Zonke kubo uphawu ukuba umntwana Albright syndrome. Iifoto kwezi amabala kungabonwa ngezantsi.

Nangona esi sifo neurofibromatosis, kufuneka indawo umbala "ikofu ubisi". Noko ke, Albright syndrome zibandakanya iindawo ezinkulu kunye lichaza mgaqweni, zibe mbalwa inani. Zinelungelo sangqa ukusuka ku-1 ukuya iisentimitha ezimbalwa, ithinti emdaka. Umbala ziyafana zonke, ukuba okweqanda imilo, ukuba luphawulwa kumphezulu egudileyo. Ngosuku loviwo histological rhoqo utyhila ukuba sangaphandle akawatshintshi kubume bayo, kodwa isixa-bala lintsundu kwi keratinocytes landa kancinci.

amabala akhethekileyo awodwa yale hlobo ingafunyanwa nayo ebantwini ngenye besempilweni. Ukuba ngaba ungenakuwuxhalabela kwaye musa kukhula, ngoko ke unyango ayikho imfuneko. Ukuba kukho ukukhula okukhawulezayo, kukho okudodobalisa spots ezimise, kucetyiswa ukuba baphande ngazo histologically. Kwaye emva koko yasuswa intsumpa.

isiphelo

Ngenxa yoko, sinokuthi for Albright syndrome luphawulwa ilahleko ithambo okanye yokakayi, ubukho amabala yesikhumba sakhi, ukufikisa ekuqaleni. Nangona kukho iimeko apho kukho kuphela iimpawu ezimbini zokuqala. Ngokubanzi, iphambili lesi sifo unezilonda ithambo (osteodysplasia). Nangona kunjalo, le nkqubo siyeka xa ufikisa. Xa abadala, utshintsho ithambo abayi phambili. Ngokubanzi, kukufunyanwa unyango olufanelekileyo, ALS unyango kwesi sifo kakhulu intle.