Vachiberekwa adrenal hyperplasia isiri yekenza: kuongorora chirwere uye kurapwa

Vachiberekwa adrenal hyperplasia isiri yekenza - guru-siyana masero zvirwere dzinomuka sezvo migumisiro urema mapuroteni backhaul. Munyaya ino tichataura pamusoro chii vari zvirwere izvi, uyewo nzira nechirwere uye kurapwa.

A mashoko mashoma pamusoro chirwere

The adrenal mumasero secrete imwe mhando chaiye mahormone, zviri kuitika munyika dzinotsanagura kukura uye muviri ukure wose. Nemamwe mashoko, vachiberekwa adrenal dysfunction kunzi adrenogenital urwere kana adrenocortical hyperplasia isiri yekenza.

Saka, chimiro pathologies adrenal boka iri kazhinji chokuita vasanyanye cortisol. Izviwo, ari kukonzera wakawedzera secretion pamusoro adrenocorticotropic kemikari. Saka, varwere ndaona kukura chinokosha thickening pamusoro adrenal cortex.

The dambudziko hosha munyika yanhasi

Vachiberekwa dysfunction of adrenal cortex - chirwere iri nhaka. Uyezve, zvokurwara ichi chinoitika zvakaenzana maitikiro mune vakadzi uye mune varume. Kune akasiyana akawanda mumwe chiremba chirwere, uye akapotsa mumwe wavo hakupindirani noupenyu. Ndicho chikonzero vana vane chirwere ichi kazhinji kufira pakuzvarwa.

Sezvinoratidzwa zvokurapa uwandu, vachiberekwa adrenal hyperplasia isiri yekenza rinowanzoberekera somugumisiro urema ari maviri okutanga hydroxylase. Uyewo, kuwanda hosha kunobva nhangemutange. Somuenzaniso, kana ukawana vamiririri nhangemutange chena, kuti chirwere ichi achaonekwa ari mwana mumwe 14 zviuru. Nepo Eskimos aizova kurwara ose mazana maviri namakumi masere mwana.

Sezvineiwo, nhasi dambudziko yokubata zvirwere izvi zviri kugadziriswa zvachose, asi chete kupfuura dzakabudirira munyika. Pamusoro kuonekwa chirwere ichi kuchangotanga vakashinga vachiitisa zvidzidzo chaiwo kubatsira kuziva vachiberekwa chirwere sezvo mangwanani sezvinobvira. Saka, zvinokwanisika here kutanga zvakarurama vachiwirirana hormone kurapwa pamberi kutanga zviratidzo adrenal insufficiency.

Zvinhu zvinowedzera mikana

Kazhinji, vachiberekwa adrenal hyperplasia isiri yekenza kunoitika kana chirwere akawanikwa kanenge mubereki, kana kana vabereki vari vatakuri zvakananga Gene nokuda dzaifanoiswa pamusoro mapuroteni kuti aifanira kubudisa adrenocortical mahormone. Kana akadaro zviratidzo yakawanikwa vabereki vose, pane mukana maviri nezvishanu kubva muzana mbudzana kuti, zvokurwara ichi dzakaonekwa.

Iyo inosvitsa kukura dysfunction

Zvakakosha kunzwisisa sei zvinofambira mashandiro pamusoro adrenal hyperplasia isiri yekenza. It kusvetuka kuti somugumisiro kukodzera chokufambisa mapuroteni kuchaita kuderera uwandu adrenal aldosterone uye cortisol. Izvi zvinokonzera kuwedzera kugadzirwa adrenocorticotropic kemikari pedyo pituitary shwanu. kemikari Iri basa yokudzora zvakakodzera nokushanda adrenal mumasero pachavo.

Kana adrenocorticotropic kemikari anobudiswa zvakanyanyisa guru kwazvo, izvi zvinotungamirira kuna chinonakidza kuti hyperplasia isiri yekenza. Zvichakubatsirawo kuonekwa zvakawedzera secretion kwevarume mahormone. A guru Kuwanda androgens kunotungamirira deformation pakati sikarudzi, uyewo kunyanya bvudzi kukura kumeso uye zvose pamusoro muviri wake.

marudzi chirwere

In okurapa, pane vatatu siyana chirwere:

1. Nyore, kana virility chimiro. chimiro ichi kazhinji kunoitika apo duku mapuroteni kushaikwa-maviri okutanga hydroxylase.
2. Solteryayuschaya chimiro vachiberekwa adrenal hyperplasia isiri yekenza kunoratidzwa zvikuru kushayiwa enzayemu ichi.
3. Vane chirwere cheBP kushanduka chinovapo somugumisiro yokushaya zvaikosha enzayemu-gidrolaktazy 11b.

zviratidzo

The chimiro chokutanga Kazhinji achiperekedzwa nokuvapo zviratidzo zvakadai penisoobrazny clitoris, labia guru moshonkoobraznye kana urogenital sinus mune mukadzi. In vanhu, vanhurume akaona nokukurumidza chaizvo kukura kwete chete mumuviri ndege, asiwo zvepabonde. Panguva iyoyowo vakomana vakachengeta izoseksualny mhando kukura, uye vasikana - a kwomurume.

Solteryayuschaya chimiro vachiberekwa adrenal hyperplasia isiri yekenza Kazhinji vana. Kuwedzera pamusoro apa zviratidzo vari akawedzera kune wekutanga uye zvakakura kutyorwa kuri adrenal cortex. Izvi zvinokosha ungaderedza kudya, electrolyte muviri uye asingauyi kuwedzera muviri uremu. Uyezve, kazhinji kacho zvinoita hypotension uye kupera mvura mumuviri kunoitika. Kana zviratidzo vasiri kuzivikanwa kunyange tanga chirwere, pane mukana mukuru rufu pamberi kuparara zvakavapo. Kana kubvira pakutanga kwaiva kunonga kurudyi kurapwa, zera kwakadaro rakadai rinogona kubviswa zvachose.

Vachiberekwa adrenal hyperplasia isiri yekenza vakadzi kunotungamirira nokuchinja mune nemamiriro kwokunze nhengo dzokubereka nadzo. Izvi anosunungura pakaitwa munhurume kemikari androgen asingadi kukanganisa Kusiyanisa yomunyika hadzi bonde nezvomukati. Kazhinji ovaries uye dumbu kuerera pemipata chose chakaipa. External nhengo dzokubereka nadzo dziri isina kukodzera kuumbwa pakuzvarwa vasikana. In okurapa, Makanga nyaya, kana kuzvarwa musikana zviri basa mukono chechikadzi.

Nokuda Migumisiro yakaipa androgens, varwere vanotanga kukura nokukurumidza kubva pakuberekwa. Very mangwanani vanoonekwa yechipiri zvepabonde maitiro. Uyezve, varwere vose vane nomumhu uye pachinzvimbo disproportionate nhamba. The chiuno yakamanikana chaizvo uye pamapendekete - zvakare paupamhi. Muchiitiko chino, pane vakadzi havana kutanga kukura mazamu, uye hariwaniki kumwedzi. Panguva iyoyowo, iyo kwokunze sikarudzi vari dzakachinjwa, muviri bvudzi rinowanikwa pamusoro muviri, uye izwi rake kuwana yakaderera.

Varwere Male kuva maererano izoseksualnym mhando. Nechekare mu mucheche zvinogona kuonekwa hypertrophy pakati yemukomana uye manhu uyewo kazhinji duku saizi. Varwere kuyaruka kazhinji simuka bundu inotungamirwa ari testes. Kakawanda, vanhu pamberi chirwere ichi vanotambura mbereko.

Vane chirwere cheBP chimiro chirwere chiri dzinoratidzwa zvose zviratidzo pamusoro apa, asi pamwe cheBP. Paaiita rinokosha kuongorora vanachiremba cherechedzai zvinhu zvakadai: kuvapo mapuroteni mu weti, mwoyo pamiganhu kuwedzerwa uye dzakachinjwa fundus midziyo.

The non-dzekare chimiro chirwere

The non-dzekare chimiro vachiberekwa adrenal hyperplasia isiri yekenza ndicho nyore uye munyoro weboka chirwere. Muchiitiko chino, 21-hydroxylase anogadzirwa mari zvishoma zvishoma kupfuura dzose. Panyaya iyi, kazhinji sikarudzi vari varume nevakadzi kukura zvakanaka, uye zviratidzo chirwere wokutanga zvingava pachena chete pashure kuyaruka. Kakawanda, non-dzekare chimiro vachiberekwa adrenal hyperplasia isiri yekenza kuonekwa vakadzi vari kunetseka pamusoro nezvinetso zvakadaro:

• Zvachose mhirizhonga munyika kutenderera varikutsika;
• mwero kana zvakakomba muchimiro mburwa;
• hazvibviri pamuviri;
• Dzimwe nzvimbo dziri mumuviri kuwana peganda romusoro kwevarume mhando.

Kakawanda, kurambwa zviduku, saka hapana simba pamusoro mashandiro kuberekana haripi. Women kuwana kana kuedza pamuviri kuti chirwere "vachiberekwa adrenal hyperplasia isiri yekenza". The non-dzekare kurapwa iro inoitwa chete vakadzi vane varume kazhinji regai kukonzera chero zvakakomba vachivavhiringidza. Naizvozvo, mabatiro ndiyo kukodzera.

chirwere chirwere

Zvakakosha zvikuru kuti mwana kuona kuvapo kwezvirwere zvakadai vachiberekwa adrenal hyperplasia isiri yekenza. Diagnostics kunoita kuti chaunoti chimiro chirwere, uye kunobatsira kuti kubata zvimwe kurapwa.

Vose vakaberekwa vaine anyatsoona abnormalities vari nemamiriro chokunze nhengo dzokubereka nadzo, vanachiremba vanoita chaiyo Diagnostics, izvo anobvumira chidzore bonde chromatin, uyewo kuona karyotype. Kakawanda, apo vachiberekwa muchimiro chirwere ichi muvarwere vaduku iri kuchengetwa muropa kunyanyisa chitsama murume kemikari. Kuti tizive pamwero zvakakodzera kuti tiite imwe kuongorora bvunzo. Kazhinji, kuvhenekwa kwakadaro akaratidza kuti wevasungwa kuti kemikari ichi muropa vakapfuura kanoverengeka.

Sezvo chikazozikamwa, vachiberekwa adrenal hyperplasia isiri yekenza mune vana pamwe nokukurumidza chaizvo kukura repfupa tishu. Kuti kudzidza kwayo marongerwo uye magadzirirwo, vanachiremba kushandisa nzira paku Diagnostics. Muchiitiko chino, X-ray zvaitwa, uye nechekare kubva mifananidzo tingaziva pfupa zera murwere. Kana pane chirwere, ndiwo mamiriro ose mapfundo mapfupa zera kazhinji vari kure mberi nemakore murwere.

Kana kuberekwa vasikana kuonekwa zviratidzo hermaphroditism, vanachiremba dzinoita Ultrasound kuti nhengo dzokubereka cheka. Muchiitiko chino, kazhinji mamiriro avo haana kusiyana vasingambooni chakaipa nazvo.

Uye pagore upenyu vana okutanga chiremba differential kuongorora chirwere. Kunoitawo kuti uzive dzenhema kana dzechokwadi hermaphroditism iri. Panyaya iyi, iyo chirwere nzira iri karyotyping. Uye, chokwadi, iva nechokwadi kuti kuona ufanane hono kemikari androgen.

Kunze pamusoro nzira zvakare inoongorora anamnestic mashoko. Huru uye kukosha chokuratidza anganzi pachiyero 17-hydroxyprogesterone.

Vachiberekwa adrenal hyperplasia isiri yekenza Vana vanogona kuwanika rechina kana rechishanu zuva pashure pokuberekwa achibatsirwa muitiro akadai neonatal kuongorora. Ongororo iyi zvinosanganisira vachiongorora 17-hydroxyprogesterone muropa wakatorwa kubva chitsitsinho. Ongororo iyi zvichakubatsira kuziva pamberi chirwere mumazuva okutanga muupenyu. Izvi zvichabatsira mangwanani kurapwa.

kurapwa

Vachiberekwa adrenal hyperplasia isiri yekenza, zviratidzo zviri pamusoro apa, nokuda tinozvarwa zvisikwa inofanira kubatwa kugara zvinodhaka kuzvitsinhanisa kurapa kwakadaro "prednisolone" uye ose analogues ayo. Kuramba chokuendesa glucocorticoids pamuviri rine zvakanaka simba. Kudivi rimwe, izvi zvinhu vanokwanisa kubvisa adrenal insufficiency vachiberekwa mavambo. Ukuwo, kugadzirira ine mahormone, vanokwanisa kudzvinyirira kunyanya secretion pamusoro adrenocorticotropic kemikari. Uyezve, anotanga akagadziridzwa inofambiswa mashandiro, uye saka akazoita kunonyanyisa pamwero hydro.

Kana hosha solteryayuschuyu chimiro, kuti riri dzinoratidzwa kushayikwa pamusoro mineralocorticoid, mishonga iri kuwedzera munyu kugadzirisa uye deoxycorticosterone acetate. Uyezve, vose vasikana vanodawo kuvhiya kupindira, iyo unogona kubvisa hypertrophied clitoris.

The nzira kuchinja mabatiro

Vachiberekwa adrenal hyperplasia isiri yekenza, mishonga izvo zvinogona here kukurira zvokurwara ichi, vanofanira kutanga muuduku. Izvi zvinofanira kuitwa nokukurumidza pashure chirwere yakanga dzakaonekwa.

Kakawanda, chinangwa vanachiremba vari zano kushandisa zvinodhaka ndiko "Prednisolone" yokutsiva mishonga. Rine ose anodiwa ehupfumi kuti kurapwa kwezvirwere ichi, uye yakarurama chipimo chemushonga vakasarudzwa haachina pamusoro womunhu muviri pakufurira.

marapirwo muitiro pachayo inofanira kutanga zvidzidzo chirwere nemiedzo. Muchiitiko chino, vanachiremba akarayira zvinodhaka izvi vhiki 15-20 MG rimwe zuva kana maviri - milligrams mana "Dexamethasone", iro vanofanira maviri matatu mazuva. Kana yokuedzwa iri dzakaratidza nemigumisiro yakanaka, saka unogona kutanga mushonga zvachose "prednisone". Kazhinji, nezuva chipimo chemushonga anenge 10-15 MG pazuva. Zvisinei, mumwe nomumwe nyaya iri zvachose nomumwe, saka munyaya dziri chete zvayo audzwa. It zvinoenderana nemakore mwana, uyewo nenhanho mahormone muropa uye mwero virilization.

Sezvo dose zvishoma nezvishoma wakaderedzwa pamusoro nguva. Izvi zvinoitwa 2-2.5 MG pavhiki. Kwokupedzisira chipimo chemushonga inogona vakatsunga chete pashure chete kana miviri pashure kutanga kurapwa. Maererano nenyanzvi, nezuva chipimo chemushonga vana pasi pemakore gore rimwe zvinofanira kusarudzwa chete nomumwe, maererano zvekuchipatara mashoko ari kupfuura hormone parameters. Kutaura zvazviri, kusarudza Zvakanyanya Kunaka Panguva Yepamuviri dose pakati nembudzana iwayo akaoma. Zvakakodzera kuitisa kuramba tichizviedza, kuitira kuti anzvenge ngozi ovhadhozi. zvazvingava Minimum zuva nezuva MG 2.5 chete, uye apo kunonyanyisa - 15 MG.

Yeuka kuti kushandisa zvinodhaka izvi vanofanira nguva dzose. Izvi zvakanakisisa mangwanani uye masikati, pashure zvokudya. Kana uine chido chokuchinja mushonga, zvakakodzera kufunga ayo vasiri basa.

Mune zvimwe zviitiko, mushonga "cortisone" anoshandiswa kurapa chirwere ichi. Zvisinei, hazvina kugona kupa kurukutika uye zvenguva pfupi anokonzerwa pamusoro kudzvinyirira ose adrenocorticotropic kemikari. mushonga Iri simba "Prednisolone" nguva anopfuura mashanu, uye saka zvinoda guru chipimo chemushonga zvakakwana. Babies zvinodhaka izvi kazhinji kutarisirwa intramuscular jekiseni mangwanani.

Mumwe inonyatsobudirira glucocorticoid vaiona "Dexamethasone". Anokwanisa kudzvinyirira kugadzirwa adrenocorticotropic kemikari kagumi zvakanyanya kukunda zvinoita "Prednisolone". Asi zvinokosha kufunga kuti ane anenge hapana zvazvinoita munyu muviri, uye kazhinji pave ovhadhozi. Daily wokuyera of fuma iri kazhinji 0.25-0.5 MG.

zvigadzirwa zvose pamusoro apa vachava pamusoro muviri inokanganisa chete panyaya kusarudzwa isiri chipimo chemushonga. Izvi zvinoratidza kuti kuwana mari yakawanda kupfuura kunodiwa nomuviri, vachava avhiringidza paari. Kana kunyanya kushandisa chero zvinodhaka izvi murwere chichachengetwa akatsvuka uye woita chiso, wakawedzera kudya, uye dzimwe nguva kuturika kuti kukura. Zvisinei, zvose migumisiro ovhadhozi chaizvo nokukurumidza kunyangarika kana ikakuruma yayo kuderedza, kana kune mishonga ine simba.

nzira kuvhiya kurapwa

Kazhinji, vachiberekwa adrenal hyperplasia isiri yekenza iri nhaka munzvimbo autosomal recessive mhando. Muchiitiko chino, hazvina basa kuti chimiro ane chirwere ichi.

Mumwe nzendo kurapwa vasikana mapurasitiki kuvhiyiwa. Most kazhinji akaita pakuvhiya paumbwe yakarurama chimiro labia minora, mumwe resection pakati clitoris uye hwomukova urogenital sinus. Kwakadaro kuvhiyiwa kunokosha, sezvo zvinobatsira kuti adzorere zvose yakarurama vasiri mashandiro anoita muviri vasikana, uyewo kwevanhu uye mupfungwa kukosha. Saka, mwana haafungi uduku yavo, saka unogona kupiwa kukireshi kana mitambo chikamu. Zvisinei, cherechedza kuti unofanira kuita nokushanda kuti gore mumwechete atanga kurapwa pamwe hormone nevamiririri.

Zvakakosha kudzidza zvose zvinoumba chirwere akadai vachiberekwa adrenal hyperplasia isiri yekenza. ICD-10 - remarudzi kupatsanura zvirwere umo hosha ane bumbiro E.25.0

Vachiberekwa adrenal hyperplasia isiri yekenza uye pamuviri

Kana murwere ane post-pubertal chimiro chirwere, pane mukuru mukana pakati undeveloped mudumbu, kubvisa pamuviri, nokupfupfudzika uye zvokurapa pamuviri. Kakawanda vakadzi vane pamuviri chokunyunyuta pamusoro murombo simba uye mhedziso kurasikirwa kudya. Dziripowo, uye electrolyte kusaenzana. Kazhinji chete maviri namasere vhiki pamuviri, murwere chirwere akavandudzika yekudyidzana. Iva nechokwadi kuyeuka kuti kunyange kana kuchengeta mwana haagoni kushivirira discontinuation pamusoro hormone zvinodhaka. Kuita izvi vachava upenyu.

Kusununguka, uye nguva uvatevere - izvi anonetsa nokuda muviri wose, uye yemubatanidzwa pamusoro adrenal cortex. Naizvozvo, kana chirwere "vachiberekwa adrenal hyperplasia isiri yekenza", pamusoro mamiriro utano kuongorora vanofanira kutora nguva. Mukadzi anofanira kudzora mahormone zviratidzo.

Maberere ramangwana

Virilnoe chimiro vachiberekwa adrenal hyperplasia isiri yekenza, uyewo mamwe chimiro anogona kupa zvakanaka zvakafanotaurwa chete apo pakatangira nenguva uye kurapwa. Chokwadi, zvinokosha chaizvo kuti ndisimbise zvakarurama zvokuongorora zvachose.

Kazhinji, kana nenguva kutanga kurapwa kunogona kudzivisa kuvhiringidzika nemamiriro munhukadzi kwokunze sikarudzi. Zvakakosha chaizvo kuti kusarudza chipimo chemushonga. Kana izvi zvaitwa nepasipo kumisa kukura kwomuviri, uye vakadzi masculinization nenhamba.

Vachiberekwa dysfunction pakati adrenal cortex nonclassical haisi ngozi chimiro chirwere, asi kurapwa kwacho apo vachisiya mukadzi zvichida Kusaroora.

Zvisinei, zvakakodzera kurapwa, kunyange vakadzi solteryayuschey chimiro chirwere, vane mikana yose kuti vave nepamuviri. Chinhu chikuru - usamhanya pachavo, nguva dzose vachitora chiremba ushandiswe mishonga, uye kuramba mararamiro akanaka. Zvino iwe Haazotyi upi chirwere. Utano.