Azalwa naso Hyperplasia adrenal: ukuxilongwa kanye nokwelashwa

Ukungasebenzi kahle kwama-cortex adrenal yizinhlobonhlobo zezifo ezithinta izifo ezivela ngenxa yokukhubazeka kwamakhemikhali e-protein. Kulesi sihloko sizoxoxa ukuthi izifo ezinjalo ziphi, futhi nezindlela zokuxilongwa nokuphathwa kwazo.

Amagama ambalwa ngesifo ngokwawo

I-grenal adrenal ihlanganisa uhlobo olukhethekile lwe-hormone ethatha ingxenye esebenzayo ekuthuthukisweni nasekukhuleni kwayo yonke inyama. Ngamanye amazwi, ukukhubazeka okubeletha kwe-cortex eyimithi ebizwa ngokuthi i-adrenogenital syndrome noma i-hyperplasia ye-cortex e-adrenal.

Ngakho-ke, ukubukeka kwamathambo wezinhlayiya ze-adrenal kuleli qembu kuvame ukuhambisana nokunciphisa ekukhiqizeni i-cortisol. Futhi lokhu, kuyimbangela yokukhushulwa kwe-horrenone ye-adrenocorticotropic. Ngakho-ke, iziguli zakha ukuqina okuphawulekayo kwe-cortex e-adrenal.

Inkinga yesifo ezweni lanamuhla

Ukungasebenzi kahle kwama-cortex adrenal yisifo esidluliselwa yifa. Kulesi simo, lesi sifo siyabonakala ngesilinganiso esifanayo kokubili besilisa nabesilisa. Kunezinhlobo eziningana zemitholampilo yesifo ngasinye, futhi cishe zonke zazo azihambisani nokuphila. Yingakho izingane ezinesifo lesi sifo zivame ukufa ngesikhathi sokuzalwa.

Njengoba izibalo zezokwelapha zibonisa, ukukhubazeka kokubeletha kwe-cortex eyingozi ngokuvamile kubangelwa ukutholakala kweziphambeko ku-hydroxylase engamashumi amabili nanye. Futhi ukusabalalisa kwalesi sifo kuncike emncintiswaneni. Isibonelo, uma uthatha abameleli bomncintiswano omhlophe, khona-ke lesi sifo sizobonakala emntwaneni owodwa ku-14 000. Ngesikhathi ama-Eskimos ezoba nawo wonke umuntu ogula ngamakhulu amabili nesishiyagalombili.

Ngenhlanhla, kuze kube yimanje, inkinga yokulwa nalezi zifo ikwazi ukuxazululwa, kodwa kuphela emazweni athuthukile kakhulu emhlabeni. Lapho lokhu kutholakala, kusemncane, izisebenzi zezempilo zenza izifundo ezikhethekile ezizosiza ukukhomba isifo sofuba ngokushesha ngangokunokwenzeka. Ngakho-ke, kungenzeka ukuqala ukwelashwa kwe-hormonal ekhethiwe kahle ngisho nangaphambi kokubonakala kwezimpawu zokuqala ezingenakuthola.

Izinto ezandisa ingozi

Ngokuvamile, ukungasebenzi komzimba kwe-cortex eyimvelo kuphakama uma lesi sifo sitholakale okungenani omunye wabazali, noma uma abazali bephethe izakhi zofuzo ezidingekayo ukukhiqiza ama-hormones e-cortex adrenal. Uma isakhi sofuzo sitholakala kulaba bobabili abazali, khona-ke kukhona amathuba okuba ngamaphesenti angamashumi amabili nanhlanu ukuthi umntwana uzotholakala nalesi sifo.

Okuholela ekuthuthukiseni ukungasebenzi

Kubaluleke kakhulu ukuqonda ukuthi indlela yokusebenza kabi kwe-cortex ephezulu iqala kanjani. Kufanele kucatshangelwe ukuthi ngenxa yokudluliswa okungalungile kwamaprotheni, inani le-aldosterone ne-cortisol emgodini we-adrenal lizokwehla. Futhi yilesi sizathu sokwandisa ukukhiqizwa kwe-horrenone ye-adrenocorticotropic nge-pituitary gland. Le hormone inesibopho sokulawula ukusebenza kahle kwezingqungquthela eziyingozi.

Uma ama-hormone e-adrenocorticotropic akhiqizwa ngokweqile kakhulu, khona-ke lokhu kuholela esimweni esifana ne-hyperplasia. Futhi, kuzokwanda ukwanda kwama-hormone wesilisa. I-androgens enkulu kakhulu iholela ekukhubazekeni kwezitho zobulili, kanye nokukhula ngokweqile kwezinwele ebusweni nasemzimbeni wonke.

Amafomu wesifo

Ezokwelapha, kunezinhlobo ezintathu zalesi sifo:

1. Ifomu eliyinkimbinkimbi, noma i-viril. Leli fomu ngokuvamile livela ngephutha elincane leprotheni ye-hydroxylase engamashumi amabili nanye.
2. Ifomu lokuhlunga lokungasebenzi kokubeletha kwe-cortex eliyingozi libhekene nokuntuleka okujulile kwalesi enzyme.
3. Ukuhlukahluka kwe-hypertonic kwenzeka ngenxa yokuntuleka okukhulu kwe-enzyme 11b-hydrolactase.

Izimpawu zesifo

Ifomu lokuqala livame ukuhambisana nokutholakala kwezimpawu ezifana ne-clitoris efana ne-penis, i-scrotum labia enkulu noma i-urogenital sinus emantombazaneni. Bobabili abesilisa nabesifazane bathuthukiswa ngokushesha kakhulu hhayi nje yendiza engokwenyama, kodwa futhi yezocansi. Ngesikhathi esifanayo, abafana banezinhlobo zokuthuthukiswa ngokocansi, futhi amantombazane ayenokuthuthukiswa ngokobulili obufanayo.

Ifomu lokuhlunga lokungasebenzi kokubeletha kwe-cortex adrenal ngokuvamile ibonakala ezinganeni. Ngaphandle kwezibonakaliso ezingenhla, ukukhathazeka okuqala kwe-cortex ye-adrenal nakho kwengeziwe. Ngesikhathi esifanayo, isifiso sokudla, isisombululo somzimba we-electrolyte nesisindo somzimba asikhulanga kakhulu. Ngaphezu kwalokho, ngokuvamile i-hypotension iqala futhi iyanciphisa umzimba. Uma lezi zibonakaliso azikwazanga ukubonakala ezinyathelweni zokuqala zesifo, khona-ke kukhona ingozi enkulu yokufa phambi kwezinto eziqhamukayo zokuwa. Uma ukwelashwa okulungile kukhethwe kusukela ekuqaleni, bese-ke iminyaka yobudala ezinjalo zingasuswa ngokuphelele.

Ukungasebenzi kahle kwama-cortex adrenal kubantu besifazane kuholela ekuguqulweni kwesakhiwo sezitho zangasese zangaphandle. Ngesikhathi esifanayo, inani elikhulu lamahomoni abesilisa abanjwe i-androgen ayithinti ukuhlukaniswa kwezitho zangasese zomzimba zangaphakathi. Ngokuvamile ukuthuthukiswa kwama-ovari kanye nesisu kujwayelekile. Izitho zangasese zangaphandle azimele kahle ngesikhathi sokuzalwa kwentombazane. Ezokwelapha kwakukhona amacala amaningi lapho kuzalwa intombazane esabelwe ubulili besilisa.

Ngenxa yemiphumela emibi ye-androgens, iziguli ziqala ukukhula masinyane kusukela ekuzalweni. Kakhulu kakhulu banezici zobunikazi zesibili. Ngaphezu kwalokho, zonke iziguli zinokukhula okuphansi kanye nomzimba ongafani kakhulu. Isikhumba sihlala sincane kakhulu, futhi amahlombe aphezulu kakhulu. Kulesi simo, abesilisa abaqali ukukhula nezilonda ezincelisayo, futhi ukuya esikhathini akubonakali. Ngesikhathi esifanayo, i-genitalia yangaphandle iguqule, isikhumba saso sibonakala kuwo wonke umzimba, futhi izwi liba ngaphansi.

Iziguli zamadoda zikhula ngokuvumelana nohlobo lobulili obuhlukile. Ngisho esemncane, ungabona ukuxilonga kwesitho socansi, futhi amakholomu ngokuvamile anezinga elincane. Esikhathini senkathi yobusha, iziguli zivame ukuhlakulela ama-tumorous formations kumathambo. Ngokuvamile abantu abanalesi sifo bahlukumezeka.

Ifomu legazi eliphakeme lelesi sifo libhekwa nazo zonke izimpawu ezingenhla, kodwa futhi lihambisana ne-high blood pressure. Lapho uqhuba izivivinyo ezikhethekile, odokotela baqaphela izinkomba ezinjalo: ukuba khona kwamaprotheni emcimbini, imingcele yenhliziyo ivuliwe, futhi izitsha ze-fundus ziyashintshwa.

Ifomu engavumelani nesifo

Ifomu elingavamile lokungasebenzi kokubeletha kwe-cortex eliyingozi libhekwa njengendlela elula futhi elula yenkinga yesifo. Kulesi simo, i-21-hydroxylase ikhiqizwa ngevolumu kancane kunejwayelekile. Kulesi simo, ngokuvamile izitho zobulili zombili abesilisa nabesifazane zikhula ngendlela efanele, futhi izibonakaliso zokuqala zokugula zingabonakala kuphela emva kwesikhathi sokuqala. Ngokuvamile, uhlobo lokungasebenzi olubucayi lwe-adrenal cortex lubonakala kubesifazane abathintekayo yizinkinga ezinjalo:

• Ukuphulwa okuphakade emjikelezweni wokuya esikhathini;
• Ifomu eliphakathi noma elibi le-acne;
• Awukwazi ukukhulelwa;
• Ezinye izindawo emzimbeni zithola uboya bezinwele.

Ngokuvamile, ukwehluleka akubalulekile, ngakho akukho mphumela emsebenzini wokuzala. Abesifazane bathola lapho bezama ukukhulelwa ukutholakala kwesifo "ukungasebenzi komzimba kwe-correx adrenal". Ifomu elingenayo i-nonclassical, eliphathwa kuphela kwabesifazane, ngokuvamile akubanga imbangela enkulu emadodeni. Ngakho-ke, ukwelashwa akukwaziswa.

Ukuxilongwa kwesifo

Kubaluleke kakhulu ukucacisa ukuba khona kwesifo esinjalo njengokungasebenzi kokuzalwa kwesifo se-cortex ebuntwaneni. Ukuxilonga kukuvumela ukuthi unqume uhlobo lwesifo, futhi kusize ukuqonda ukwelashwa okuqhubekayo.

Zonke izingane ezizalwa neziphazamiso ezibonakalayo ezakhiweni zobulili zangaphandle, odokotela benza ukuxilongwa okukhethekile, okukuvumela ukuba unqume ukuthi i-chromatin yesondo, futhi unqume ukuthi i-karyotype. Ngokuvamile, esimweni sokuzalwa kwalesi sifo ezinzimbeni ezincane, kunesilinganiso esiningi ngokweqile le-hormone yesilisa egazini. Ukuze unqume izinga layo, ukuhlolwa kokuhlolwa kufanele kwenziwe. Ngokuvamile ukuhlola okunjalo kubonisa ukuthi ukuhlushwa kwaleli hormone egazini kudlulelwe izikhathi eziningana.

Njengoba kuyaziwa, ukungasebenzi komzimba kwe-cortex eyingozi ezinganeni kuhambisana nokukhula okusheshayo kwamathambo esithambo. Ukuze ufunde isakhiwo nesakhiwo sayo, odokotela basebenzisa indlela yokuxilongwa kwezinto. Kulokhu, i-x-ray yenziwe, futhi kakade ngokwezithombe, iminyaka yesiguli ingaqunywa. Uma kukhona i-pathologies, ngokuvamile ngokuvamile isimo samalungu kanye namathambo ngokukhula kunesidingo ngaphambi kwesikhathi sobuguli.

Uma ekuzalweni, amantombazane abone izibonakaliso ze-hermaphroditism, khona-ke odokotela benza i-ultrasound yamalungu omzimba wesifazane. Kulokhu, isakhiwo sabo ngokuvamile asifani nesimiso.

Ngonyaka wokuqala wokuphila kwezingane, odokotela benza ukuhlonza okuhlukile. Ikuvumela ukuba unqume ukuthi i-hermaphroditism ingamanga noma ayiqiniso. Kulesi simo, indlela yokuhlonza i-karyotyping. Futhi-ke, kuyadingeka ukunquma izinga le-hormone androgen yesilisa.

Ngaphandle kwezindlela ezibalwe ngenhla, idatha ye-anamnestic ibuye ihlaziywe. Isimiso esiyinhloko nesibaluleke kakhulu singabizwa ngezinga le-17-hydroxyprogesterone.

Ukungasebenzi kahle kwama-cortex adrenal ezinganeni kungatholwa kakade ngosuku lwesine noma lwesihlanu emva kokuzalwa ngokusebenzisa inqubo efana nokuhlolwa kwezingane. Lokhu kuhlaziya kuhilela ukuhlola i-17-hydroxyprogesterone egazini elithathwe esithendeni. Ukwenza ukuhlaziywa okunjalo kuzosiza ukwazi mayelana nokutholakala kwezinkinga ezinsukwini zokuqala zokuphila. Lokhu kuzosiza ukuqala ukwelashwa ngendlela esifike ngesikhathi.

Izindlela zokwelapha

Ukungasebenzi kahle kwegciwane le-cortex eliyingozi, izimpawu zazo ezichazwe ngenhla, ngenxa yemvelo engokwemvelo, kufanele iphathwe ngelashwa oluqhubekayo lokusebenzisa izidakamizwa ezifana ne-Prednisolone, kanye nazo zonke izinhlobo ezifanayo. Ukudla njalo kwe-glucocorticoids emzimbeni kunomphumela omuhle kuye. Ngakolunye uhlangothi, lezi zinto zingakwazi ukuqeda ukungakwazi kahle kwemvelaphi yangempela. Ngakolunye uhlangothi, izidakamizwa eziqukethe ama-hormone zingakwazi ukuvimbela ukuvinjwa ngokweqile kwe-horrenone ye-adrenocorticotropic. Ngaphezu kwalokho, indlela yokuphendula iqala ukulawulwa, okusho ukuthi izinga le-hydrogeneration linciphiswa.

Uma lesi sifo sinesimo sokulahlekelwa usawoti, okungukuthi, sibonakala nokuntuleka kwemineralocorticoids, khona-ke izixazululo zikasawoti kanye ne-acetate ye-deoxycorticosterone nazo zengezwe ekwelapheni. Ngaphezu kwalokho, wonke amantombazane adinga ukungenelela okuhlinzekwa, okungasusa i-clitoris ye-hypertrophic.

Indlela yokwelashwa okulondolozayo

Ukungasebenzi kahle kwama-cortex adrenal, okwelashwa okungabhekana nalesi sifo, kufanele kuqale kusukela ebuntwaneni. Yenza lokhu ngokushesha ngemuva kokuba lesi sifo sibonwe.

Ngokuvamile, ngenhloso yokwelashwa endaweni, odokotela bayalwa ukuba basebenzise lesi sidakamizwa "Prednisolone". Unayo zonke izakhiwo ezidingekayo zokwelashwa kwalesi sifo, futhi nesilinganiso esifanele ngeke sibe nomthelela omubi emzimbeni womuntu.

Yona kanye inqubo yokwelapha kufanele iqale ngokuhlolwa kokuhlola. Kulolu daba, odokotela banikeze lesi sidakamizwa ngesonto ngesigidi esingu-15-20 mg ngosuku noma ama-milligram amabili "Dexamethasone", okufanele athathwe ezinsukwini ezimbili kuya kwezintathu. Uma lokhu kuhlolwa kubonisa imiphumela emihle, ungaqala ukwelapha unomphela nge-Prednisolone. Ngokuvamile, umthamo wansuku zonke uwu-10-15 mg ngosuku. Noma kunjalo, icala ngalinye lingumuntu ngamunye, ngakho-ke lesi sihloko siqukethe izincomo ezijwayelekile kuphela. Kuxhomeke eminyakeni yobudala, kanye nasezingeni lamahomoni egazini nasezingeni eliphakeme le-virilization.

Ngokuhamba kwesikhathi, umthamo uyancipha kancane kancane. Lokhu kwenziwa ku-2-2.5 mg ngesonto. Isilinganiso sokugcina singanquma eyodwa kuphela noma izinyanga ezimbili emva kokuqala kokwelashwa. Ngokusho kochwepheshe, ukulinganisa nsuku zonke izingane ezingaphansi kweminyaka eyodwa kufanele zikhethwe ngabanye kuphela, zigxile ngesikhathi esifanayo kwimininingwane yomtholampilo ngaphezu kwemingcele ye-hormonal. Eqinisweni, ukukhetha umthamo omuhle wengane kunzima kakhulu. Kubalulekile ukuqhuba ukuhlolwa njalo ukuze kuqedwe ingozi yokudlula ngokweqile. Isibonelelo esincane nsuku zonke si-2.5 mg kuphela, kanti ubukhulu buyi-15 mg.

Sicela uqaphele ukuthi udinga ukuthatha le mithi njalo. Kungcono ukukwenza ekuseni nangentambama, emva kokudla. Uma udinga ukuguqula umuthi, kufanele uqaphele umsebenzi wayo wokuphila.

Kwezinye izimo, umuthi "Cortisone" usetshenziselwa ukwelapha lesi sifo. Kodwa-ke, iyakwazi ukuba nomthelela obuthakathaka futhi omfishane ekunciphiseni ukwenziwa kwamahomoni adrenocorticotropic. Lesi sidakamizwa sibuthakathaka kune-Prednisolone izikhathi ezingaphezu kwesisihlanu, ngakho kudinga isilinganiso esincane kakhulu. Izinsana ezinjalo izidakamizwa zivame ukuphathwa nge-intramuscularly ekuseni.

Enye ye-glucocorticoids esebenzayo kakhulu i-dexamethasone. Iyakwazi ukuvimbela ukukhiqizwa kwe-hormone ye-adrenocorticotropic izikhathi eziyishumi ngokuphumelelayo kune-Prednisolone. Kodwa kubalulekile ukucabangela ukuthi cishe akukho nhlobo emthonjeni wosawoti, futhi ngokuvamile uvusa ukudlula ngokweqile. Isimiso sezinsuku zonke salesi sifo ngokuvamile si-0.25-0.5 mg.

Zonke izidakamizwa ezingenhla ziyoba nomthelela omubi emzimbeni kuphela uma ukhetha isilinganiso esingalungile. Lokhu kusikisela ukuthi ukuthatha isidakamizwa okungaphezu komzimba kudinga kuyoba nomthelela omubi kuyo. Ngokusebenzisa ngokweqile noma yikuphi kwalezi zidakamizwa, isiguli sizokwazi ukubona ama-hypermia nokuzihlanganisa ubuso, ukukhula kwesidlo, futhi ngezinye izikhathi ngisho nokuma ekukhuleni nasekuthuthukisweni. Noma kunjalo, yonke imiphumela yokudlula ngokweqile izoshabalalisa ngokushesha uma iyancishiswa kancane, noma iya emtholampilo obuthakathaka.

Indlela yokwelapha ukwelashwa

Ngokuvamile ukungasebenzi komzimba kwe-cortex eyimvelo kuzuzwa i-autosomal uhlobo oluphindaphindiwe. Akukhathaleki ukuthi ngabe lesi sifo sinjani.

Esinye sezigaba zokuphathwa kahle kwamantombazane ukuhlinzeka nge-plastic. Ngokuvamile, ukungenelela okuhlinzekwayo kwenziwa ukwenza ifomu elifanele le-labia minora, ukuthengiswa kwe-clitoris nokuvulwa kwesono se-urogenital. Ukusebenza okunjalo kuyimpoqo, njengoba kusiza ukubuyisela yonke imisebenzi efanele yokusebenza komzimba wentombazana, futhi kubaluleke kakhulu emphakathini nangokwengqondo. Ngakho-ke, i-kid ayizizwa ingaphansi kwayo, ngakho-ke inganikwa inkulisa noma isigaba sezemidlalo. Nokho, sicela uqaphele ukuthi udinga ukwenza umsebenzi okungenani unyaka owodwa emva kokuqala kokwelashwa ngezidakamizwa ze-hormone.

Kubalulekile ukufunda zonke izici sifo ezifana azalwa naso Hyperplasia adrenal. ICD-10 - i ngezigaba international izifo lapho lesi sifo has a E.25.0 ikhodi

Azalwa naso Hyperplasia adrenal nokukhulelwa

Uma isiguli unomzimba post-pubertal lesi sifo, kukhona ematfuba aphezulu fetus ongakasetshenzwa, isisu, kwesisu kanye kwezisu wezokwelapha. Isikhathi esining impela abesifazane abakhulelwe esekhala ubungqabavu abampofu futhi ukulahlekelwa ngokuphelele ukudla. Hhayi ngaphandle, futhi electrolyte ukungalingani. Ngokuvamile nge sonto amabili nesishiyagalombili ka ukukhulelwa, isimo lesiguli ngcono kakhulu. Qiniseka khumbula ukuthi ngisho nalapho okuvikela umntwana abakwazi ukubekezelela ukuqedwa izidakamizwa hormone. Ukwenza lokhu kuyodingeka ukuphila.

Zokubeletha, futhi isikhathi emva kwabo - lena kucindezele kakhulu kuwo wonke umzimba, futhi sekukhona nezinhlelo eziningi cortex adrenal. Ngakho-ke, lapho isifo "azalwa naso Hyperplasia adrenal", isimo ukuhlaziywa impilo kuzodingeka ukuba uzinike isikhathi. Umuntu wesifazane kufanele ukulawula izinkomba hormone.

Izinhlelo lekusasa

Virilnoe uhlobo azalwa naso Hyperplasia adrenal, kanye nanoma yimuphi omunye ifomu anganika izibikezelo sihle kuphela uma kuqale ukwelashwa esifike ngesikhathi futhi efanele. Yiqiniso, kubaluleke kakhulu ukusungula ngokuphelele uphethwe yini.

Ngokuvamile, lapho isiqalo ngesikhathi esifanele ukwelashwa kungavimbela yokuphazamiseka isakhiwo insikazi zangaphandle izitho zangasese. Kubalulekile kakhulu ukukhetha Isikali lesifanele. Uma lokhu kwenziwa ngendlela engafanele, kuzoyeka ukukhula komzimba, nabesifazane ngeke masculinization izibalo.

ukungasebenzi azalwa naso we adrenal cortex nonclassical akuyona ifomu njengoba eyingozi kakhulu lesi sifo, kodwa inqubo yokwelapha ngenkathi uziba owesifazane kungenzeka angabi nangane.

Nokho, ukwelashwa okufanele, ngisho abesifazane nge ifomu solteryayuschey lesi sifo, unawo wonke amathuba ukuze ayakhulelwa. Into esemqoka - musa ukusebenzisa ngokwabo, njalo ethatha udokotela ebekiwe zemithi ethengiswayo, futhi bakwazi ukuphila okuhle. Futhi ngakho-ke angeke wesabe lutho isifo. Yiba enempilo.