Bekhubazekile hyperplasia adrenal: isifo kwaye afumane unyango

Bekhubazekile hyperplasia adrenal - iintlobo ngentlobo ukuphazamiseka yemfuza ezivela ngenxa iziphene protein WiMAX. Kweli nqaku siza kuthetha ntoni na ezi zifo, ngokunjalo iindlela loxilongo kunye nonyango.

A amagama ambalwa malunga nesifo

Le adrenal cell ukuba uhlobo olulodwa emzimbeni, athathe inxaxheba kwiinkqubo zophuhliso kunye nokukhula umzimba uphela. Ngamanye amazwi, kwekhono naso adrenal wabhekisela syndrome njengoko adrenogenital okanye hyperplasia adrenocortical.

Ngoko ke, ukuvela ezimbi adrenal eli qela lidla ezinxulumene ekuncipheni cortisol. Oku, ebangela secretion olongeziweyo hormone adrenocorticotropic. Ngenxa yoko, izigulane ziye ubone ubhekiso phambili ukuqina ebalulekileyo uthinte adrenal.

Ingxaki kwesi sifo kwihlabathi lanamhlanje

kwekhono azalwa le neuron adrenal - sisifo into njengelifa. Ngapha koko, le ngezifo kwenzeka rhoqo ngokulinganayo amabhinqa kunye into eyindoda. Kukho iintlobo ezininzi izifo ngamnye kwezonyango, yaye phantse zonke kubo akuvumelani ebomini. Yiloo nto abantwana nesi sifo badla ndingafeli embilinini.

Njengoko kuboniswe manani zonyango, hyperplasia naso adrenal kaninzi kwenzeka ngenxa ziintsilelo hydroxylase ezingamashumi amabini ananye. Kwakhona, ukwanda kwesi sifo ixhomekeke kugqatso. Umzekelo, ukuba uthatha abameli abamhlophe, ukuba esi sifo kuya kuthathwa omnye umntwana ka-14 amawaka. Nangona Eskimos wayeza kuba ogulayo lalingamakhulu amabini anamanci asibhozo yonke usana.

Ngethamsanqa, namhlanje ingxaki wokujongana ezi zifo kusonjululwa ngokupheleleyo, kodwa kuphela kumazwe ahambele phambili ehlabathini. Xa efumanise esi sifo nangoku paramedics elisezantsi ukuqhuba izifundo ezizodwa ukunceda ekuboneni yesifo azalwa kwangethuba kangangoko kunokwenzeka. Ngaloo ndlela, kuyenzeka ukuba ukuqala unyango ufanise ngokuchanekileyo kwamadlala phambi uhlaselo iimpawu insufficiency adrenal.

Imiba ezandisa amathuba

Kwiimeko ezininzi, hyperplasia naso adrenal kwenzeka ukuba isifo wafunyanwa umzali omnye ubuncinane, okanye ukuba abazali abathwali gene ngqo lothutho iiproteni ukuba eziyimfuneko ukuze kufezekiswe iihomoni adrenocortical. Ukuba isakhi onjalo eyafumanekayo ngabazali bobabini, kukho amathuba amabini anesihlanu ekhulwini kid, le ngezifo ibanjiwe.

Leyo ekhokelela kuphuhliso kwekhono

Kubalulekile ukuqonda ukuba ihamba njani na indlela hyperplasia adrenal. Kubangel 'umdla ukwazi ukuba ngenxa iiprotheni zothutho emihle kuya kuncipha ubungakanani aldosterone adrenal kunye cortisol. Oku kubangela ukwanda yi ingqondo yebhinqa kwimveliso incindi yedlala adrenocorticotropic. Le hormone inoxanduva lokulawula ukusebenza olufanelekileyo adrenal ngokwabo.

Ukuba hormone adrenocorticotropic eziveliswa yanikwa ngokugqithiseleyo, oku kukhokelela le ngxaki yokusetyenziswa hyperplasia. Lo mgaqo uya kubonakala secretion olongeziweyo iihomoni eyindoda. A ndzo elikhulu androgens kukhokelela deformation ye zangasese, kwakunye nokukhula iinwele kakhulu kwi ebusweni bonke phezu komzimba wakhe.

Isifo

Xa yezonyango, kukho iintlobo ezintathu sifo:

1. Ayinzima, okanye ifomu virility. Le fom ngokuqhelekileyo kwenzeka xa iproteni intswelo encinane hydroxylase yamashumi amabini ananye.
2. Solteryayuschaya uhlobo azalwa hyperplasia adrenal iphawulwa ukuhluleka olunzulu yale enzyme.
3. kuvela ukwahluka unesifo ngenxa yokunqongophala kakhulu enzyme-gidrolaktazy 11b.

iimpawu

Ifomu yokuqala sidla sikhatshwe ubukho iimpawu ezifana penisoobrazny ikhozo, kwiLibia moshonkoobraznye enkulu okanye sinus enzala amabhinqa. Xa abantu, oyindoda noyinkazana wabona uphuhliso olukhawulezayo kakhulu kuphela moya umzimba, kodwa ngokwesondo. Ngelo xesha linye amakhwenkwe wabona uhlobo izoseksualny yophuhliso, kunye namantombazana - a yindoda nebhinqa.

Solteryayuschaya uhlobo hyperplasia naso adrenal ngokuqhelekileyo kubonwa ebantwaneni. Ukongeza kule mpawu zingasentla nazo wongezelela kowokuqala kunye nokuhlukunyezwa esinzulu le uthinte adrenal. Oku kunciphisa kakhulu ukutya, electrolyte ukutya kunye nokutha emzimbeni ayinyuki. Ukongeza, ngokufuthi nto iphuhlisa hypotension emzimbeni kwenzeka. Ukuba iimpawu aziboniswanga phaya nkqu nisaqala zesi sifo, kukho ingozi enkulu yokufa phambi iziganeko yokuwa. Ukuba ukusuka kanye ekuqaleni ukuba ukukhetha unyango ekunene, ubudala loo nesenzeko lupheliswe ngokupheleleyo.

hyperplasia adrenal bekhubazekile kwabasetyhini kukhokelela utshintsho isakhiwo esini lwangaphandle. Oku ukhupha imali enkulu okwendoda hormone androgen aluchaphazeli umahluko ngamalungu angaphakathi sex ababhinqileyo. Ngokwesiqhelo, amaqanda kunye abapasileyo isibeleko flow eqhelekileyo ngokupheleleyo. esini bangaphandle zisuke luyasekwa amantombazana ekuzalweni. Xa yezonyango, kwakukho eninzi amatyala, xa ukuzalwa intombazana kokuba unikwe yesini eyindoda.

Ngenxa yeziphumo elibi androgens, izigulane ziqala ukukhula ngokukhawuleza kakhulu ukusuka ekuzalweni. Kakhulu ekuqaleni ke kuvela neempawu zesini eziziisekondari. Ukongeza, zonke izigulane kufuneka elifutshane ngesiqu, nani kunokuba omkhulu. Isinqe libanzi kakhulu kwaye amagxa - ngokubanzi kakhulu. Kulo mzekelo, amabhinqa ayizange iqalise ukukhula amabele, yaye akafuni kubonakala sexesheni. Kwangaxeshanye, amalungu obuni lwangaphandle ilungiswe, uboya umzimba ivela emzimbeni, kwaye ilizwi lakhe uya asezantsi.

izigulane Male zikhula njengoko uhlobo izoseksualnym. Sele ebuntwaneni inokubonwa hypertrophy lwe lobudoda kunye namasende zisoloko abe ubukhulu encinane. Xa izigulane ekhula rhoqo kuvela ithumba uluhlu amatyhalarha. Amaxesha amaninzi, abantu phambi kwesi sifo ubunzima ngenxa ukungachumi.

uhlobo unesifo esi sifo linophawu zonke mpawu ezidweliswe ngasentla, kodwa ephelekwa uxinzelelo oluphezulu lwegazi. Ekuqhubeni uphando ezikhethekileyo oogqirha isaziso ezo zinto: ubukho iiprothini umchamo, imida intliziyo landiswa kunye neempahla fundus ilungiswe.

I non-classical uhlobo sifo

Le fom non-classical of hyperplasia naso adrenal likhondo kakhulu elula ndithantamisa esi sifo. Kulo mzekelo, 21-hydroxylase evela imali ngaphantsi kancinci nje eqhelekileyo. Kulo mzekelo, ngokuqhelekileyo amalungu esini xa amadoda nabafazi ukuphuhlisa kakuhle, kwaye imiqondiso yokuqala sifo kuba kubonakala kuphela emva elivisayo. Amaninzi, non-classical uhlobo hyperplasia adrenal naso ibonwe abasetyhini abathe ukhathazekile malunga neengxaki ezifana:

• ukuphazamiseka abasisigxina menses kumjikelo;
• uhlobo eliphakathi okanye kakhulu induna;
• akunakwenzeka ukuba ukukhulelwa;
• Ezinye iindawo kwi umzimba bafumana Nentloko enesihlwitha uhlobo eyindoda.

Amaninzi, minor zilahliwe, ngoko azinampembelelo umsebenzi zizale ayinikezi. Amabhinqa ukufumana xa uzama ukukhulelwa ngenxa yesifo "hyperplasia azalwa adrenal". I non-classical uhlobo unyango esiqhutywa kuphela abafazi yinkunzi ngokuqhelekileyo musani esingenakubangela naliphi na ukuphazamiseka kakhulu. Ngoko ke, unyango yihambo engafanelekanga.

lokufunyaniswa kwesifo

Kubaluleke kakhulu ukuba umntwana ukufumanisa ubukho izifo ezifana hyperplasia naso adrenal. Uxilongo ikuvumela ukuba ukuchaza uhlobo sifo, yaye inceda ukujongana unyango olungaphezulu.

Bonke abantwana abazelwe ukungaqheleki ngokuqaphelekayo ubume esini yangaphandle, oogqirha ukwenza uxilongo ezizodwa, nto leyo evumela ukuba ukuchaza chromatin ngesondo, kwakunye ukugqiba karyotype. Amaninzi, xa uhlobo naso esi sifo kwizigulane abaselula liyasetyenziswa egazini isixa ngokugqithisileyo hormone eyindoda. Ukuqinisekisa inqanaba eziyimfuneko ukwenza uvavanyo ukuhlolwa. Ngokuqhelekileyo, uvavanyo olo lubonisa ukuba xi kwale hormone egazini ngaphaya izihlandlo eziliqela.

Njengoko yaziwa, hyperplasia adrenal iziphene kubantwana lukhatshwa kukukhula ngokukhawuleza kakhulu esiqwini ithambo. Ukuze ukufunda isakhiwo sayo kunye nemvakalo, oogqirha basebenzisa indlela kuxilongo instrumental. Kulo mzekelo, kwenziwe X-reyi, kwaye sele evela imifanekiso sikwazi ubudala ithambo isigulane. Ukuba kukho isifo, oko kudla ngumqathango onke amalungu kunye nobudala bone zikude ngaphambi kweminyaka yobudala umguli.

Ukuba ukuzalwa amantombazane abonakale iimpawu hermaphroditism, oogqirha enze ultrasound zangasese. Kulo mzekelo, ubume babo ngokuqhelekileyo akukho kwahluka kwisiqhelo.

Kwakhona ngomnyaka wokuqala iintsana ubomi ugqirha diagnosis umahluko. It ikuvumela ukuba ukuchonga hermaphroditism bobuxoki okanye bokwenyaniso. Kulo mzekelo, indlela diagnostic into karyotyping. Kakade ke, qiniseka ukuba ukujonga umgangatho hormone androgen eyindoda.

Ngaphandle kwezi ndlela kwakhona ngokuhlalutya data anamnestic. Isibaluli kwaye ibalulekileyo ekunokuthiwa umgangatho-hydroxyprogesterone 17.

hyperplasia adrenal bekhubazekile ebantwaneni ingafunyanwa ngomhla wesine okanye sesihlanu emva kokuzalwa ngoncedo inkqubo ezifana zokuhlola lweentsana. Olu hlahlelo ibandakanya ukutshekishwa-hydroxyprogesterone 17 egazini losana isithende. Olu hlahlelo kuya kunceda ukuchonga ubukho sifo ngemihla yokuqala yobomi. Oku kuya kunceda unyango kwangoko.

ayini

hyperplasia azalwa adrenal, iimpawu ezo zichazwe ngasentla, ngenxa yobume ongaphakathi kufuneka ziphathwe kwanonyango rhoqo endaweni iziyobisi ezifana 'prednisolone "kwaye ukuhlela analogue yayo. ukunikezelwa rhoqo glucocorticoids emzimbeni inamandla nempembelelo entle kakhulu. Kwelinye icala, ezi izinto banako ukunciphisa insufficiency adrenal imvelaphi naso. Kwelinye icala, amalungiselelo eziqulathe emzimbeni, bayakwazi ukucinezela secretion ngokugqithisileyo hormone adrenocorticotropic. Ukongezelela, iqala indlela ingxelo ehlengahlengisiweyo, kwaye yiyo kunciphisa kwinqanaba ubuninzi ophehlwa.

Ukuba sifo solteryayuschuyu ifom, ukuba linophawu kukho ukuswela le mineralocorticoid, unyango nako kongezwa izisombululo ityuwa deoxycorticosterone acetate. Ukongeza, onke amantombazana kufuneka ungenelelo ngotyando, ngalo uyakwazi ukususa i ikhozo hypertrophied.

Indlela unyango elondoloza

hyperplasia azalwa adrenal, unyango ongayi ngenene ukumelana nale ngezifo, kufuneka luqale ebuntwaneni. Oku kufuneka kwenziwe ngoko nangoko emva kokuba sifo kubonakele.

Amaninzi, injongo oogqirha bacetyiswa ukuba basebenzise i yi "Prednisolone" replacement therapy iziyobisi. Inazo zonke iipropati eziyimfuneko ukuze unyango lwesi sifo, kwaye idosi echanekileyo okhethiweyo akayi kuba emzimbeni womntu zinempembelelo embi.

inkqubo yonyango ngokwalo kufuneka iqale ngokuqhuba diagnostic tests. Kulo mzekelo, oogqirha amisele eli chiza ukuba kwiveki ka 15-20 mg usuku okanye iintsuku ezimbini - milligrams ezine "Dexamethasone", ekufuneka kuthabatha iintsuku ezimbini ukuya kwezintathu. Ukuba le uvavanyo lubonise iziphumo ezilungileyo, ukuze ungakwazi ukuqala unyango esisigxina "prednisone". Kwiimeko ezininzi, idosi yemihla ngemihla imalunga 10-15 mg ngosuku. Nangona kunjalo, kwimeko nganye nakanjani ngamnye, ngokunjalo kwinqaku zidweliswe iingcebiso ngokubanzi kuphela. Kuxhomekeke yobudala yomntwana, kwakunye kwinqanaba hormone egazini kunye neqondo virilization.

Ekubeni ithamo ngokuthe yehliswe kwexesha. Oku kwenziwa 2-2.5 mg ngeveki. Umthamo zokugqibela kumiselwa iinyanga kuphela emva enye okanye ezimbini emva kokuqala unyango. Ngokutsho kweengcali, idosi yemihla ngemihla kubantwana abangaphantsi kweminyaka konyaka omnye kufuneka zikhethwe ngamnye kuphela, ngokuhambelana data lwezonyango kwi ngaphezu hormone iparameters. Enyanisweni, ukukhetha idosi ngokupheleleyo itakane kunzima kakhulu. Kuyimfuneko ukuba ukuqhuba uvavanyo oluqhubekayo, ukuze Ukunqanda umngcipheko libanda. izinga Ubuncinane ngemihla mg 2.5 kuphela, kwaye ngelixa ubuninzi - 15 mg.

Khumbula ukuba ukuthatha la machiza kufanele njalo. Oku kwenziwa ngcono kusasa nasemva kwemini, emva kwesidlo. Ukuba unayo imfuneko ukutshintsha ichiza, kuyimfuneko ukuba siqwalasele umsebenzi wayo nokomzimba.

Kwezinye iimeko, ichiza "cortisone" asetyenziswa ukunyanga esi sifo. Noko ke, ukuba uyakwazi ukunika iziphumo incipha elifutshane phezu kwezithandani incindi yedlala adrenocorticotropic. Eli chiza ithambile "Prednisolone" izihlandlo ezingaphezu kwesihlanu, kwaye ngoko ke ifuna idosi enkulu ngokwaneleyo. Iintsana la machiza esuke ngenaliti intramuscular kusasa.

Enye glucocorticoid kakhulu njenge "Dexamethasone". Uyakwazi ukucinezela imveliso hormone adrenocorticotropic izihlandlo ezilishumi ngobuchule kunokuba "Prednisolone". Kodwa kuyimfuneko ukuba luthathele ingqalelo ukuba sele phantse akukho alunasiphumo kwi ukutya ityuwa, yaye ngokufuthi negalelo i libanda. kwiingxowa Daily osisiqu jikelele 0.25-0.5 mg.

Yonke imveliso ezidweliswe ngasentla liya kuba phezu emzimbeni nefuthe elibi kuphela kwimeko yokukhetha idosi engalunganga. Oku kubonisa ukuba uthatha imali ngaphezu kufuneka yi ngumzimba, kuya kuba yingozi kuye. Ukuba ukusetyenziswa ngokugqithisileyo naziphi na kwezi machiza isigulana siya babonwe ububomvu kunye ubuso ajikelezayo, zokuphila, yaye ngamanye amaxesha ukunqunyanyiswa yokukhula kunye nophuhliso. Nangona kunjalo, zonke iziphumo ze-libanda kakhulu shwaka ngokukhawuleza xa bit yayo ukunciphisa, okanye bafudukele imithi esibuthathaka.

indlela yonyango steel

Ngokuqhelekileyo, hyperplasia naso adrenal into njengelifa kulo autosomal uhlobo sofuzo. Kulo mzekelo, akunamsebenzi oko fomu esi sifo.

Enye amanqanaba unyango olufanelekileyo mantombazane uqhaqho zeplastiki. Amaninzi kwenziwe utyando kwi ukuyilwa uhlobo esichanekileyo minora kwiLibia, kukho resection le ikhozo kunye ukuvulwa sinus enzala. Olo tyando umele, njengoko kunceda ukubuyisela yonke imisebenzi komzimba elichanekileyo kwezi ntombi zomzimba, kwakunye ziyakubaluleka kwezentlalo nasengqondweni. Ngoko ke, umntwana angacingi ukuba kwabo phansi, ngoko ke kunikwa abancinane okanye icandelo kwezemidlalo. Nangona kunjalo, qaphela ukuba kufuneka enze umsebenzi zawo ubuncinane isithuba sonyaka omnye emva kokuqalisa unyango kunye arhente hormone.

Kubaluleke kakhulu ukufundisisa zonke iinkalo zesifo, ezinjenge-congenital disysction of corrox adrenal. I-ICD 10 ikwahlula ngezizwe ngezifo apho esi sifo sinekhowudi E.25.0

Ukukhulelwa komzimba kunye nokukhulelwa

Ukuba isigulane sinesimo sengxelo esiphezulu sokusasazeka kwesifo, ngoko kukho ukuphakama okuphezulu komntwana ongaphangeliyo, ukukhulelwa kwesisu, ukukhulelwa kwesisu nokukhupha isisu. Abafazi abaninzi abakhulelweyo bayakhononda ngobuthathaka kunye nokulahlekelwa ngumdla. Ibhalansi ye-electrolyte ayifakiwe. Ngokuqhelekileyo, kwiiveki ezingamashumi amabini anesibhozo zokukhulelwa, imeko yesigulane iphuculwe kakhulu. Qiniseka ukuba uqaphele ukuba nangona uthabathe umntwana, ungavumeli ukupheliswa kokuthatha imithi ye-hormonal. Yenza oku kubomi.

Ijonga kunye nexesha elilandelayo emva kwazo lixinzeleleke kakhulu kwimizimba yonke, kunye ne-corréx ene-adrenal ebandakanya. Ngoko ke, ukuba isifo "sisifo sokuzalwa komntwana," ukuhlaziywa kwemoya yempilo kuyakufuneka ihanjiswe rhoqo. Ibhinqa limele ilinge ama-hormone.

Iiprojekti zekamva

Ifom ye-viral ye-congrenital adrenal distection, kunye nayiphi na enye ifomu, inokunika ubungqina obuhle kuphela xa kuqaliswa unyango olufanelekileyo ngexesha elifanelekileyo. Ewe, kubaluleke kakhulu ukuseka ukuxilongwa ngokuchanekileyo.

Ngokuqhelekileyo ngokuqala ngexesha lokonyango, kunokwenzeka ukukhusela ukuphulwa kwindlela yokwenziwa kwamalungu esini angaphandle. Kubaluleke kakhulu ukukhetha ixabiso elifanelekileyo. Ukuba oku kwenziwa ngokungalunganga, ngoko ukukhula komzimba kuya kumisa, kwaye ukuxhaswa komzimba kweso siza kubakho kwabasetyhini.

I-congenital adrenal i-cortex i-dysfunction ayilona uhlobo oluqhelekileyo lwesifo eso, kodwa ukuba uyayinakuyinyamekela inkqubo yokonyango, umfazi unobungozi bokungabantwana.

Nangona kunjalo, ngo kunyango olufanelekileyo, kwanamabhinqa aphethwe yityuwa-ukulahlekelwa kweso sifo banamathuba okukhulelwa. Into ephambili - musa ukuzibamba, uhlale uthatha izidakamizwa ezichanekileyo, kwaye uholele ubomi obuhle. Kwaye awusayi kuba nesifo na. Yimpilo.