Kune hutano hwepabonde uye hukuru hwepabonde, mahomoni ane mhosva, mamwe acho anobudiswa muzvinyorwa zvinonzi adrenal. Kune chirwere chakaberekwa chinoratidzirwa nekusavhiringidza kwezvipembenene zve endocrine nekuwedzerwa kwakanyanya kwe androgens. Kuwedzerwa kwemashure ehurume pabonde mumuviri kunounza kuchinja kukuru muurongwa hwemuviri.
Adrenogenital Syndrome - Zvinokonzera
Ihwo hutachiona hunenge huri kuongororwa hunobva kuhutano hwekuberekwa hupenyu hunotora nhaka. Izvo zvisingawanzoongororwa, chiitiko cheAdrenogenital Syndrome chikamu 1 pa 5000-6500. Kushandurwa kweiyo genetic code kunokonzera kuwedzera kwehukuru nekuderera kwe adrenal cortex. Kubudiswa kwemakemikari anokosha anobatanidzwa mukugadzirwa kwe cortisol uye aldosterone kunopera. Kushayikwa kwavo kunotungamirira ku kuwedzera kwehutachiona hwemadhimoni evanhukadzi.
Adrenogenital syndrome - classification
Zvichienderana nehuwandu hwehuwandu hwekuwedzera kwekuwedzera uye kuoma kwezviratidzo, iyo yakatsanangurwa chirwere iripo mune zvakasiyana-siyana. Mafomu eAdrenogenital syndrome:
- munyu-kurasikirwa;
- viril (nyore);
- post-pubertal (nonclassical, atypical).
Adrenogenital syndrome - mhando yemunyu
Rudzi rwakawanda kwazvo rwehutachiona, runowanikwa mune zvitsva kana vana vegore rokutanga rehupenyu. Nenzira yekurasikirwa nemunyu yeadrenogenital syndrome, iyo inonzi hormonal balance inoshungurudzwa uye basa reAdrenal cortex harina kukwana. Urwu urwere hwechirwere hunofambidzana nehutsika hwakanyanya hwema aldosterone. Izvo zvakakosha kuchengetedza mvura yemunyu muyero. Iyi adrenogenital syndrome inokonzera kukanganisa kwebasa remwoyo uye kusvetuka muropa. Izvi zvinopesana nechekupedzisira kwekuunganidza masarti muitsvo.
Adrenogenital syndrome is viol form
Kusiyana kwakajeka kana kwechikoro chechikwata chehutachiona hakusi pamwe nezviitiko zvekuda kwekuwedzera. Iyo yakatsanangurwa adrenogenital syndrome (ACS viril fomu) inotungamirira kungoshanduka kune maitiro ekunze. Urwu urwere hwehutachiona hunoonekwawo mudiki kana kuti mushure mekuberekwa. Mukati rehutano hunoramba huripo.
Postpubertate maitiro eAdrenogenital syndrome
Urwu urwere hwehutano hunonziwo atypical, inowanikwa uye isiri-classical. Utachiona hwakadaro hune adrenogenital hunoitika kune vakadzi chete vane hutano hwakanaka hwepabonde. Chinokonzera kukura kwehutachiona chinogona kuva chepenital mutation yejeni, uye chiremera cheAdrenal cortex . Chirwere ichi chinowanzofambidzana nekushaya simba, saka pasina chirwere chakakwana, adrenogenital syndrome uye kuzvitakura hazvienderani. Kunyangwe nekubudirira pamuviri, njodzi yekuparara pamuviri yakakwirira, fetus inourayiwa kunyange mumatangi ekutanga (mavhiki manomwe kusvika ku10).
Adrenogenital syndrome - zviratidzo
Chirwere chemakiriniki chechirevo chakatsanangurwa chema genetic chinofanana nezera uye chimiro chechirwere. Adrenogenital syndrome muzvizvarwa zvitsva dzimwe nguva hazvigoni kutariswa, nekuda kwei izvo zvepabonde zvemucheche zvinogona kuona zvisakarurama. Zviratidzo zvakajeka zvehutachiona zvinoonekwa kubva pamakore 2-4, pane dzimwe nguva zvinoratidzira gare gare, mukuyaruka kana mukukura.
Adrenogenital syndrome muvakomana
Nemunyu-kurasikirwa kwechimiro chechirwere, zviratidzo zvemvura-munyu zvinokonzera kukanganiswa kunoonekwa:
- diarrhea;
- kurutsa kukuru;
- kuderera kwemuviri weropa;
- kunetseka;
- tachycardia;
- kuora muviri.
A simple adrenogenital syndrome muvana vane zviratidzo zvinotevera:
- kuwedzera penis;
- kunyanyisa ganda rekugadzirwa kwechando;
- giza epidermis chakakomberedza anus.
Vacheche vachangobva kuberekwa havawanzoongororwa nokuti chirwere chechipatara achiri mudiki hachina kuratidzirwa. Gare gare (kubva kumakore maviri) adrenogenital syndrome inonyatsooneka:
- kukura kwevhudzi pamuviri, kusanganisira mazamu;
- inzwi rakaderera, rine ruzha;
- hormonal acne ( acne vulgaris);
- masculinization;
- kukurumidza kwefupa maitiro;
- short stature.
Adrenogenital syndrome muvasikana
Kufananidza chirwere chinotaridzirwa mumadzimai echeche chiri nyore, chinowirirana nezviratidzo zvakadaro:
- hypertrophic clitoris, iyo inotarisa zvikuru sepenis;
- guru guru, rinotarisa sechando;
- hutachiona uye urethra zvakabatanidzwa muchivi cheurogenital.
Mukurwisana nemamiriro ezviratidzo zvevecheche, vasikana dzimwe nguva vanokanganisa nevechikomana uye vakakurira maererano nehutano hwakashata. Nemhaka yeizvi, kuchikoro kana mukuyaruka, vana ava vanowanzove nezvinetso zvepfungwa. Mukati rehutano hwemusikana wacho zvachose hunofananidzwa nejenotype yevakadzi, ndicho chikonzero iye anonzwa ari mukadzi. Mwana anotanga kupesana kwepamberi uye matambudziko nekugadzirisa munharaunda.
Pashure pemakore maviri, congenital adrenogenital syndrome inoratidzwa nezviratidzo zvinotevera:
- kukura nguva isati yakwana yebvudzi rinoputika uye mazamu;
- makumbo mapfupi nemaoko;
- muscularity;
- kuonekwa kwebvudzi rechiso (nezera remakore 8);
- muviri wemunhu (zvakapamhidza mapepa, mapapiro akapfupi);
- kusavapo kwekukura kwezvinyoro zvinyoro;
- kukura kwakadzika uye torsi huru;
- rude voice;
- acne disease;
- kutanga kunonoka kwekuenda kumwedzi (hakuna kumbopfuura makore 15-16);
- kusagadzikana kufamba, kazhinji kunonoka pakuenda kumwedzi;
- amenorrhea kana oligomenorrhea;
- kusabereka;
- ropa rinokwira;
- kunyanya kuvepa kwepidermis.
Adrenogenital syndrome - kuongororwa
Instrumental uye laboratory zvidzidzo zvinobatsira kuziva hyperplasia uye kusagadzikana kwe adrenal cortex. Kuti uongorore adrenogenital congenital syndrome mucheche, kuongororwa kwemashure edzimba uye computed tomography (kana ultrasound) inoitwa. Kuongorora kwehutori kunogona kuona mavhavha uye chibereko mukati mevasikana vane nhengo dzebereki dzechikadzi.
Kuti kusimbise zvinonzi kufungidzirwa, unyanzvi hwekuongorora hutachiona hweAdrenogenital syndrome hunoitwa. Inosanganisira kuongorora urine neropa pane zvinyorwa zvehomoni:
- 17-OH-progesterone;
- aldosterone;
- cortisol;
- 17-ketosteroids.
Kuwedzera kugoverwa:
- biochemistry ;
- kuchipatara ropa test;
- kunyanya kuongorora urine.
Kurapa kweAdrenogenital Syndrome
Hazvibviri kubvisa kuongororwa kwema genetic pathology, asi zviratidzo zvaro zvekurapa zvinogona kubviswa. Adrenogenital syndrome - kliniki mazano:
- Upenyu hwekuwana hupenyu hwemadhimoni. Kuti kuve nekugadzirisa basa reAdrenal cortex uye kutarisa endocrine muyero, iwe unoda kugara uchinwa glucocorticoids. Izvo zvinosarudzwa ndezvokuti Dexamethasone. Nhamba inotarirwa mumwe nomumwe uye inosvika kubva ku 0.05 kusvika ku2,55 mg pazuva. Nemunyu-kurasikirwa kwechimiro chechirwere, zvinokosha kutora mineral corticoid kuitira kuchengetedza mvura yemunyu.
- Kururamiswa kwechitarisiko. Varwere vane inorondedzerwa kuongororwa vanokurudzirwa kuti vave nepurasitiki yepineti, clitorectomy uye mamwe maitiro ekuvhiya kuti ave nechokwadi chokuti mazita edzimba ane maitiro akanaka uye mashizha.
- Nguva dzose kutaurirana nemunhu wepfungwa (pakukumbira). Vamwe varwere vanoda rubatsiro rwekugadzirisa vanhu uye kugamuchirwa pachavo sevanhu vakazara.
- Kukurudzirwa kweivulation. Vakadzi vanoda kutora pamuviri vanofanira kuendeswa kune imwe nzira yemishonga inoshandiswa iyo inovimbisa kururamiswa kwekuenda kumwedzi uye kubviswa kwe androgen yekugadzirwa. Glucocorticoids inotorwa nguva yose yekugumira.