Kobadwa nako adrenal hyperplasia: matenda ndi mankhwala

Kobadwa nako adrenal hyperplasia - lalikulu zosiyanasiyana matenda majini amene angapeze monga chotsatira cha kupunduka mapuloteni backhaul. M'nkhani ino tikambirana kodi matenda amenewa, komanso njira matenda ndi mankhwala.

mawu ochepa A za matendawa

The zopangitsa adrenal secrete wapadera mahomoni, ndi kukhala wochitachita mu njira ya chitukuko ndi kukula kwa thupi lonse. M'mawu ena, kobadwa nako adrenal kukanika amatchedwa matenda monga adrenogenital kapena adrenocortical hyperplasia.

Kotero, maonekedwe a pathologies adrenal gulu ili amaonera kuchepa tochepa. Zimenezi, amachititsa kuchuluka katulutsidwe wa timadzi adrenocorticotropic. Choncho, odwala aona chitukuko cha thickening yofunika kwambiri kotekisi adrenal.

Vuto la matenda mu dziko lamakono

Kobadwa nako kukanika kwa adrenal kotekisi - matenda amene tinatengera. Komanso, kudwala izi zimachitika ndi pafupipafupi ofanana akazi ndi amuna. Pali mitundu ingapo ya lililonse matenda ovutika ndi pafupifupi aliyense wa iwo ndi yosemphana ndi moyo. Ndicho chifukwa ana ndi matenda kawirikawiri kufa akangobadwa.

Malinga ndi ziwerengero zachipatala, kobadwa nako adrenal hyperplasia zambiri kumachitika chifukwa cha kupunduka mu hydroxylase makumi awiri oyamba. Komanso kuchuluka kwa matenda chimadalira pa mpikisano. Mwachitsanzo, ngati inu kutenga anthu a mtundu woyera, kuti matenda sanaonerere kukhazikitsa mwana mmodzi wa 14 chikwi. Ngakhale Eskimos akhodza kudwala iliyonse mazana awiri ndi makumi asanu ndi atatu mwana.

Mwamwayi, lero vuto zolimbana ndi matenda awa kwathunthu kuthetsedwa, koma mayiko ambiri otukuka a dziko. Pa kudziwika matenda ndi kutangoyamba akuchipatala ake kuchititsa maphunziro apadera kuthandiza kuzindikira matenda obadwa mwamsanga. Choncho, n'zotheka kuyamba molondola chikufanana mankhwala m'thupi pamaso pa isanayambike zizindikiro za insufficiency adrenal.

Zinthu zimene zingachititse chiopsezo

Nthawi zambiri, kobadwa nako adrenal hyperplasia kumachitika ngati matenda anapezeka kholo imodzi, kapena ngati makolo ali ndi jini mwachindunji kwa polumikizira mapulotini amene yoyenera kuti apange mahomoni adrenocortical. Ngati jini chinapezeka mwa makolo onse, pali kuthekera peresenti twente-faifi a mwana kuti, kudwala izi wapezeka.

Imbaenda chitukuko cha kukanika

Nkofunika kumvetsa mmene zimakhalira limagwirira wa hyperplasia adrenal. Dziwani kuti chifukwa zoyendera zosayenera mapuloteni, adzakhala ndichepe kuchuluka kwa aldosterone adrenal ndi tochepa. Zimenezi zimachititsa ponso mu ulimi wa timadzi adrenocorticotropic ndi pituitary England. timadzi Ichi ndi udindo malamulo kugwira bwino ntchito ya zopangitsa adrenal okha.

Ngati adrenocorticotropic timadzi opangidwa wambiri kwambiri lalikulu, zimenezi zimapangitsa kuti chodabwitsa hyperplasia. Izo kuona kuchuluka katulutsidwe mahomoni wamwamuna. A ndende yaikulu ya androgens kumabweretsa mapindikidwe wa maliseche, komanso kwambiri kukula tsitsi pa nkhope ndi lonse thupi lake.

mitundu ya matenda

Mu yochizira matenda, pali njira zitatu za matendawa:

1. Wopepuka, kapena mtundu virility. fomu iyi zambiri kumachitika pamene yaing'ono mapuloteni akusowa hydroxylase makumi awiri oyamba.
2. Solteryayuschaya mawonekedwe kobadwa nako adrenal hyperplasia amakhala ndi akusowa kwambiri za puloteni imeneyi.
3. Hypertensive kusintha ukapezeka chifukwa cha kupanda chidwi enzyme-gidrolaktazy 11b.

zizindikiro

Mawonekedwe woyamba kuchita zambiri ndi kukhalapo zizindikiro monga penisoobrazny nkongo, labia lalikulu moshonkoobraznye kapena urogenital nkusani akazi. Aliyense, mwamuna ndi mkazi anawona chitukuko mofulumira kwambiri osati ndege thupi, komanso kugonana. Pa nthawi yomweyo anyamatawo anaona mtundu izoseksualny chitukuko, ndi atsikana - ndi mwamuna ndi mkazi.

Solteryayuschaya mawonekedwe kobadwa nako adrenal hyperplasia zambiri ena mwa ana. Kuwonjezera zizindikiro pamwamba zapadera woyamba ndi kuswa kwambiri wa kotekisi adrenal. Izi kwambiri utachepa kudya, electrolyte kagayidwe ndipo alibe kuwonjezera kulemera kwa thupi. Komanso, nthawi zambiri akufotokozera hypotension ndi ludzu amapezeka. Ngati zizindikiro ndi losadziwika ngakhale mu magawo oyambirira a matenda, pali chiopsezo chachikulu cha imfa pamaso pa zochitika kugwa. Ngati kuchokera pachiyambi anali kukatenga mankhwala lamanja, m'badwo zochitika akhoza kwathunthu inathetsedwa.

Kobadwa nako adrenal hyperplasia mu akazi kumabweretsa kusintha kapangidwe wa maliseche kunja. Izi akumasula kwakukulu kuchuluka kwa timadzi androgen mwamuna sakhala bwanji zosiyanasiyana za ziwalo wamkazi. Mwachibadwa, thumba losunga mazira ndi chiberekero mipita otaya ndi sankaganiza. maliseche kunja ndi yosayenera anapanga pa atsikana kubadwa. Mu yochizira matenda, panali zambiri milandu, mtsikana pobadwa, iwo anasankha jenda wamwamuna.

Chifukwa mavuto obwera chifukwa cha androgens, odwala kuyamba kukula mofulumira kwambiri ndi kubadwa kwambiri. m'banda iwo amaoneka sekondale makhalidwe kugonana. Komanso, odwala onse ndi aafupi ndi m'malo akanagwiritsa chithunzi. Mafupa a chiuno ndi yopapatiza ndipo mapewa - kwambiri lonse. Pankhaniyi, akazi sanayambe kukula mabere, ndipo sizimawoneka kusamba. Pa nthawi yomweyo, kunja ziwalo zoberekera ndi kusinthidwa, thupi tsitsi limapezeka pa thupi, ndipo mawu ake kutsikira.

odwala Male kukhala malinga ndi mtundu izoseksualnym. Kale mu ukhanda Tingaone hypertrophy kwa mbolo maliseche komanso kawirikawiri ndi kukula yaing'ono. Odwala unyamata zambiri uka chotupa lomenyera testes lapansi. Nthawi zambiri, anthu pamaso pa matenda amadwala osabereka.

mawonekedwe Hypertensive matenda nazonso yodziwika ndi zizindikiro zonse zimene tanena, komanso limodzi ndi kuthamanga kwa magazi. Pogwira Kafukufuku chapadera madokotala zindikirani zinthu: kukhalapo kwa mapuloteni mu mkodzo, malire mtima kukodzedwa ndi kusinthidwa ziwiya fundus.

The sanali chakale mawonekedwe a matenda

Mawonekedwe sanali chakale cha kobadwa nako adrenal hyperplasia ndi njira ambiri kumodzi ndi wofatsa matendawa. Pankhaniyi, 21 hydroxylase kamapangikira ndalama pang'ono zosakwana yachibadwa. Pankhaniyi, zambiri kumaliseche ndi amuna komanso akazi amakula bwino ndipo zizindikiro woyamba wa matendawa akhoza kukhala zowonekeratu pokhapokha unyamata. Nthawi zambiri, si chakale mawonekedwe kobadwa nako adrenal hyperplasia ndinawona azimayi amene akukhudzidwa za mavuto amenewa:

• msokonezo okhazikika menses mkombero;
• odziletsa kwambiri kapena mtundu wa ziphuphu zakumaso;
• sikutheka mimba;
• M'madera ena pa thupi kukhala ndi khungu la mtundu wamwamuna.

Nthawi zambiri, kukanidwa wamng'ono, palibe chikoka pa ntchito ya kubereka ana sasamalira. Akazi kupeza pamene kuyesera kutenga mimba kwa matenda "kobadwa nako adrenal hyperplasia". The sanali chakale mtundu wa chithandizo imene imachitika okha akazi amene amuna ambiri samasankha chifukwa chisokonezo iliyonse yaikulu. Choncho, mankhwala ndi khalidwe zosayenera.

matenda a matenda

Nkofunika kuti mwana kudziwa pamaso pa matenda monga kobadwa nako adrenal hyperplasia. Diagnostics limakupatsani kuthandauza mawonekedwe a matenda, komanso zimathandiza kuthana ndi chithandizo zina.

ana onse amabadwa ndi kuvutika zikusintha kapangidwe wa maliseche kunja, madokotala kuchita diagnostics wapadera, amene amalola kuthandauza kugonana chromatin, komanso kudziwa karyotype. Nthawi zambiri, pamene kobadwa nako mawonekedwe a matenda odwala wamng'ono zimawonedwa mwazi wa mtengo kwambiri lalikulu la timadzi wamwamuna. Pofuna kudziwa mlingo zofunika kuchita mayeso mosamala. Childs, kuyezetsa ngati zasonyeza kuti anthu ambiri timadzi izi magazi kuposa kangapo.

Monga amadziwika, kobadwa nako adrenal hyperplasia ana limodzi ndi kukula mofulumira kwambiri minofu fupa. Pofuna kuphunzira matabwa ake ndi kapangidwe, madokotala amagwiritsa ntchito njira diagnostics malangizo. Pankhaniyi, ndi X-ray anachita, ndi kale kwa mafano tingadziwire fupa zaka wodwalayo. Ngati pali matenda, nthawi zambiri ndi chikhalidwe zimfundo onse ndi zaka fupa ali zotsogola m'badwo wa wodwalayo.

Ngati kubadwa kwa atsikana atawona zizindikiro za hermaphroditism, madokotala achite ultrasound wa aziwadula maliseche. Pankhaniyi, kapangidwe awo ndi zambiri sikusiyana mwakale.

Komanso mu chaka choyamba cha ana moyo dokotala matenda masiyanidwe. Timatha kuzindikira onyenga zoona hermaphroditism ndi. Mu nkhani iyi, njira yodziwira ndi karyotyping. Ndipo, ndithudi, onetsetsani kudziwa mlingo wa mwamuna timadzi androgen.

Kuwonjezera njira pamwamba komanso kusanthula deta anamnestic. Waukulu ndi zofunika kwambiri muyezo angatchedwe mlingo wa 17 hydroxyprogesterone.

Kobadwa nako adrenal hyperplasia ana angapezeke pa tsiku lachinayi kapena chachisanu atabadwa mothandizidwa ndi ndondomeko monga kuyezetsa magazi neonatal. kusanthula Zimenezi zimaphatikizapo afufuze 17 hydroxyprogesterone mu magazi otengedwa chidendene. kusanthula izi zidzathandiza kudziwa pamaso pa matenda mu masiku oyamba a moyo. Izi zidzathandiza chithandizo mwamsanga.

zochiritsira

Kobadwa nako adrenal hyperplasia, zizindikiro zimene zafotokozedwa pamwambapa, chifukwa chikhalidwe titadziwa kusamala nazo zonse mankhwala M'MALO mankhwala monga "prednisolone" ndipo Mitundu analogues ake. yobereka apitirize glucocorticoids mu thupi Kumakhudza zabwino kwambiri. Pa dzanja limodzi, zinthu izi ndi okhoza kuthetsa adrenal insufficiency kobadwa nako chiyambi. Komano, kukonzekera munali mahomoni, amatha kubisa katulutsidwe kwambiri wa timadzi adrenocorticotropic. Komanso akuyamba owonjezera a maganizo limagwirira, choncho kumachepetsa mlingo pazipita hydro.

Ngati matenda ndi mawonekedwe solteryayuschuyu, lomwe ndi khalidwe la kuperewera kwa mineralocorticoid ndi, mankhwala ndi anawonjezera zothetsera mchere ndi deoxycorticosterone nthochi. Komanso atsikana onse amafunika alowererepo opaleshoni, mungathe kuchotsa nkongo hypertrophied.

The njira ya mankhwala ndiwofatsa

Kobadwa nako adrenal hyperplasia, mankhwala amene angathe kwenikweni kupirira kudwala imeneyi, ayenera kuyamba ana. Izi zichitike nthawi yomweyo pambuyo matenda anali wapezeka.

Nthawi zambiri, cholinga cha madokotala akulangizidwa kugwiritsa ntchito mankhwala ndi "Prednisolone" m'malo mankhwala. Ilo liri ndi katundu zonse zofunikira zochizira matenda, ndi mlingo zolondola anasankha sadzakhala ndi pa thupi la munthu kuchita zoipa.

ndondomeko chithandizo chiyenera kuyamba ndi kuchititsa mayesero matenda. Pankhaniyi, madokotala mankhwala mankhwala kwa sabata la 15-20 mg tsiku kapena awiri - mamiligalamu anayi "Dexamethasone", amene ayenera kutenga masiku awiri kapena atatu. Ngati kuyezetsa izi zasonyeza bwino, kotero inu mukhoza kuyamba mankhwala okhazikika "prednisone". Nthawi zambiri, mlingo tsiku ndi za 10-15 mg wa pa tsiku. Komabe, nkhani iliyonse ndi munthu mwamtheradi, kotero m'nkhani ali yekha malangizo ambiri. Izo zimatengera zaka mwana, komanso pa mlingo wa mahomoni m'magazi ndi digiri ya virilization.

Popeza mlingo pang'onopang'ono mpaka kusanduka pa nthawi. Izi zachitika mwa mg wa 2-2.5 pa sabata. Mlingo komaliza zingadziŵike miyezi pambuyo mmodzi kapena awiri pambuyo kuyambira chithandizo. Malinga ndi akatswiri, mlingo tsiku ana osakwana zaka chaka chimodzi ayenera kusankhidwa payekha yekha, mu mzere ndi deta matenda pa kuposa magawo m'thupi. Ndipotu, kusankha mlingo mulingo woyenera wa mwana wa mbuzi ndi kovuta. M'pofunika kuchita kuyezetsa ankatero pofuna kupewa ngozi cha mankhwala osokoneza bongo. Osachepera tsiku mlingo wa mg wa 2.5 okha, ndipo pamene pazipita - 15 mg.

Kumbukirani kuti kumwa mankhwala awa ayenera nthawi zonse. Izi bwino anachita m'mawa ndi madzulo, pambuyo chakudya. Ngati muli ndi kusintha mankhwala, m'pofunika tione zimene zake zokhudza thupi.

Nthawi zina, mankhwala "cortisone" amagwiritsidwa ntchito pofuna kuchiza matenda. Komabe, anatha kupereka zotsatira wofooka yochepa pa yimitsira wa timadzi adrenocorticotropic. Izi mankhwala ndi yochepa "Prednisolone" maulendo asanu, choncho amafuna lalikulu mulingo okwanira. Ana mankhwala zambiri yake mu mnofu jekeseni m'mawa.

Mmodzi wa glucocorticoid yabwino kwambiri kuti "Dexamethasone". Iye akhoza kuletsa kupanga timadzi adrenocorticotropic efficiently kakhumi kuposa momwe amawonekera "Prednisolone". Koma m'pofunika kuganizira kuti ali pafupifupi ziribe mphamvu pa kagayidwe mchere, ndipo nthawi zambiri zimathandiza kuti mankhwala osokoneza bongo. Daily ponseponse wa thunthu zambiri 0.25-0.5 mg.

Zonsezi tanena adzakhala pa thupi zoyipa zokha pa nkhani ya kusankha mlingo cholakwika. Izi zikusonyeza kuti kuphunzira ndalama kuposa chofunika mwa thupi, adzakhala ndi mavuto aakulu pa iye. Ngati ntchito yambiri pa aliyense wa mankhwala wodwala udzachitika redness ndi anamaliza nkhope, kuchuluka njala, ndipo nthawizina kuyimitsidwa kukula ndi chitukuko. Komabe, onse obwera chifukwa cha mankhwala osokoneza bongo kwambiri mofulumira kutha ngati pang'ono ake kuchepetsa, kapena kusuntha kwa mankhwala chofooka.

njira ya mankhwala opaleshoni

Kawirikawiri, kobadwa nako adrenal hyperplasia ndi tinatengera mu autosomal recessive mtundu. Pankhaniyi, izo ziribe kanthu zimene mawonekedwe ali matenda.

Imodzi mwa magawo wa mankhwalawa atsikana ndi pulasitiki opaleshoni. Ambiri zambiri opaleshoni pa mapangidwe mawonekedwe lolondola la labia minora, ndi resection wa nkongo ndi kutsegula kwa nkusani urogenital. Chotero opareshoni ndi ayenera, monga zimathandiza kuti abwezeretse yolondola ntchito thupi lonse atsikana thupi, komanso kufunika chikhalidwe ndi maganizo. Choncho, mwana asaone ngati onyozeka awo, choncho angathe kuperekedwa sukulu ya mkaka kapena gawo masewera. Komabe, dziwani kuti mufunika kuchita ntchito kwa chaka chimodzi pambuyo chiyambi cha mankhwala ndi wothandizila m'thupi.

Nkofunika kuphunzira mbali zonse za matenda monga kobadwa nako adrenal hyperplasia. ICD-10 - ndi gulu lonse la matenda imene matendawa ali ndi kachidindo E.25.0

Kobadwa nako adrenal hyperplasia ndi mimba

Ngati wodwalayo pambuyo pubertal mawonekedwe a matenda, pali mkulu Mwina wa mwana wosabadwayo chimanga chosakhwima, mimba padera ndi mimba zachipatala. Nthawi zambiri amayi apakati amadandaula umoyo osauka ndi imfa mtheradi kudya. Osati lilibe, ndipo electrolyte vutoli. Kawirikawiri ndi twente chitatu sabata mimba, alili bwino kwambiri. Onetsetsani kukumbukira kuti ngakhale pamene kusunga mwana sangakhoze kulekerera discontinuation mankhwala m'thupi. Kuchita zimenezi adzakhala ndi moyo.

Kubereka, ndi nthawi pambuyo pawo - izi ndi zopanikiza kwambiri thupi lonse, ndipo Ponena za kotekisi adrenal. Choncho, pamene matenda "kobadwa nako adrenal hyperplasia", pa boma la kusanthula thanzi ayenera kutenga nthawi. Mkazi ayenera kulamulira mahomoni zizindikiro.

Kafukufuku m'tsogolo

Virilnoe mawonekedwe kobadwa nako adrenal hyperplasia, ndipo m'njira ina iliyonse angapereke zolosera yabwino pokhapokha ngati chiyambi cha panthawi yake ndiponso moyenera mankhwala. Kumene, ndikofunika kukhazikitsa matenda zolondola kwathunthu.

Nthawi zambiri, pamene yake chiyambi cha mankhwala chingalepheretse chisokonezo kapangidwe ka mkazi ziwalo kunja maliseche. Nkofunika kusankha mlingo zolondola. Ngati zimenezi molondola, isiya kukula kwa thupi, ndi akazi masculinization kanjedza.

Kobadwa nako kukanika kwa adrenal kotekisi nonclassical si oopsa mawonekedwe a matenda, koma chithandizo ndondomeko pamene kunyalanyaza mkazi ayenera kukhala wopanda mwana.

Komabe, ndi mankhwalawa, ngakhale akazi ndi mawonekedwe solteryayuschey wa matenda, ndi mwayi wokhala ndi pakati. Chinthu chachikulu - samayendetsa okha, nthaŵi zonse dokotala Kwalamulidwa mankhwala, ndi moyo wabwino. Ndiyeno inu Musaope matenda aliwonse. Kukhala wathanzi.