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I-Cushing Syndrome

Uhlolojikelele

I-Cushing syndrome iyenzeka uma umzimba unamazinga aphezulu ehormone i-cortisol isikhathi eside. Lokhu kungenzeka ngenxa yokuthi umzimba ukhulula i-cortisol eningi kakhulu, noma ngenxa yokusebenzisa imithi ebizwa ngokuthi yi-glucocorticoids, enomphumela ofanayo nomzimba njenge-cortisol. I-cortisol eningi kakhulu ingabangela ezinye zezimpawu eziyinhloko ze-Cushing syndrome — i-hump enamafutha phakathi kwamahlombe, ubuso obuqondiwe, kanye nemibhalo emhlophe noma ebomvu olukhulu olusendilini. I-Cushing syndrome ingabangela futhi ukuphakama kokucindezela kwegazi noma ukulahleka kwamathambo. Ngezinye izikhathi, ingabangela i-type 2 diabetes. Ukwelashwa kwe-Cushing syndrome kunganciphisa amazinga e-cortisol emzimbeni futhi kuthuthukise izimpawu. Uma ukwelashwa kuqala ngokushesha, amathuba okuphulukisa aba ngcono.

Izimpawu

Izimpawu ze-Cushing syndrome zingase zihluke kuye ngokwezinga le-cortisol engeziwe. Ukukhuluphala emzimbeni, ngamakhono nama-leg amathin. Ukukhuluphala ebusweni. Lokhu ngezinye izikhathi kubizwa ngokuthi ubuso lwenyanga. I-lump enamafutha phakathi kwamahlombe. Lokhu kungabhekwa njenge-buffalo hump. Izimpawu ezibomvu noma eziphuzi esiswini, ezinqenqemeni, emathangeni, ebeleni nasezandleni. Isikhumba esincinyane, esibuthakathaka esilula ukulunywa. Ukululama kwezindawo ezilimele okuhamba kancane. Ukukhula kwamafinyila. Iinwele ezicwebezelayo, ezimnyama ebusweni nasezimweni. Lesi simo sibizwa ngokuthi i-hirsutism. Izikhathi ezingavamile noma eziyeka. Ukunciphisa ukulangazelela ubulili. Ukunciphisa ukukhulelwa. Izinkinga zokufinyelela ekuqinisweni. Ukukhathala okukhulu. Ubuthakathaka bemisipha. Usizi, ukukhathazeka nokuqwasha. Imizwelo enzima ukuyilawula. Ukuphazamiseka ekuqondeni noma ukukhumbula. Ukungalali. Ukuphuma kwengcindezi yegazi. I-Headache. Ukutheleleka. Ukukhanya kwesikhumba. Ukwehliswa kwama-bone, okungabangela ukuphazamiseka kwama-bone. Ukukhula okuyimfihlo kubantwana. Shaya udokotela wakho uma unezimpawu ze-Cushing syndrome, ikakhulukazi uma uthatha imithi ye-glucocorticoid ukwelapha inkinga yempilo enjengokuqina, isifo samathambo noma isifo sokuvuvukala esibilisini.

Isikhathi sokubona udokotela

Shaya udokotela wakho uma unezimpawu ze-Cushing syndrome, ikakhulukazi uma uthatha imithi ye-glucocorticoid ukuze welaphe inkinga yezempilo enjengoburhulu, isifo samathambo noma isifo sokuvuvukala esizathwini.

Izimbangela

Uhlelo lokukhipha ama-hormone e-adrenal gland luhlanganisa ama-adrenal glands ne-pituitary gland. Izimpawu ezivela ku-pituitary gland zitshela ama-adrenal glands ukuba enze i-cortisol. I-pituitary gland iyazi uma umzimba unamazinga afanele we-cortisol.

Cushing syndrome ibangelwa ukuba nokuningi kwe-cortisol emzimbeni. I-cortisol uhormone eyenziwa yi-adrenal glands. Isizwa umzimba ukuthi uphendule ekuhluphekeni futhi idlala indima ebalulekile, okuhlanganisa:

  • Ukunciphisa ukuvuvukala.
  • Ukusizwa inhliziyo nemiphunga ukuba isebenze kahle.
  • Ukulawula ushukela egazini.
  • Ukusizwa umzimba ukuba usebenzise ukudla ukuze uthole amandla.

Cushing syndrome ingahle ibe khona ngokusebenzisa imithi ye-glucocorticoid. Le mithi ivame ukusetshenziswa ekwelapheni izifo zokuvuvukala ezinjengobhubhane lwe-rheumatoid, i-lupus ne-asthma. Ubuhlungu noma ukulimala emuva noma ezinhlangothini kanye nezinhlobo eziningi zesikhumba kungase kuphathwe nge-glucocorticoids. Zingase futhi zisetshenziswe ukuvimbela umzimba ukuba ungayenqabi inhlangano entsha ngemuva kokufakelwa.

I-Glucocorticoids ingase ithathe ngomlomo, inikezwe njengesiphuzo, igqotshelwe esikhumbeni noma iphefumlelwe emaphaphwini nge-inhaler. Noma iyiphi indlela ye-glucocorticoid, uma ithathwe ngobuningi isikhathi eside, ingabangela i-Cushing syndrome.

Uhormone owenziwe yi-pituitary gland ulawula ukuthi umzimba wenze kangakanani i-cortisol. Lokhu kubizwa nge-adrenocorticotropic hormone (ACTH). Ezinye izigwaca zenziwa yi-ACTH, okuyenza i-cortisol eningi futhi ingabangela i-Cushing syndrome. Izinkinga nge-adrenal glands zingathinta i-cortisol futhi zibangele i-Cushing syndrome.

Uma i-Cushing syndrome iyenzeka ngale ndlela, ingase ibangelwe yi:

  • I-ACTH-producing pituitary adenoma. Ama-pituitary adenomas ayizigwaca ezikhula ku-pituitary gland. Atholakala phansi kwengqondo futhi ngokuvamile awusiye umdlavuza. Lezi zigwaca ngezinye izikhathi zenze i-ACTH eningi. Lokhu kubangela ama-adrenal glands ukuba enze i-cortisol eyengeziwe. Uma i-Cushing syndrome iyenzeka ngale ndlela, kubizwa nge-Cushing disease. Kwenzeka kakhulu kwabesifazane futhi yilona uhlobo oluvamile lwe-endogenous Cushing syndrome.
  • Ectopic ACTH-producing tumor. Ngokungavamile, isigwaca esenza i-ACTH eningi sikhulayo esithombeni esingavamile ukwenza i-ACTH. Lokhu kubizwa ngokuthi ukukhiqizwa kwe-ectopic ACTH. Kubangela umzimba ukuba wenze i-cortisol eningi. Lezi zigwaca zingaba yomdlavuza, kodwa azikho njalo. Zitholakala ngokuvamile emaphaphwini, i-pancreas, i-thyroid noma i-thymus gland.
  • Ama-adrenal gland tumors noma isifo. Izinkinga nge-adrenal glands zingabangela ukuba zenze i-cortisol eningi. Okuvamile kakhulu yisigwaca esingxenyeni yangaphandle ye-adrenal gland ebizwa nge-adrenal adenoma. Lezi zigwaca azilona umdlavuza, futhi ezimbalwa kuphela ezenza i-cortisol eningi.

Izigwaca zomdlavuza ezingxenyeni zangaphandle ze-adrenal glands, ezibizwa nge-adrenocortical carcinoma, zimbalwa. Kodwa zingenza i-cortisol futhi zibangele i-Cushing syndrome. Ngezinye izikhathi, izicucu eziningi ezenza i-cortisol zingakhulayo e-adrenal glands futhi zibangele i-Cushing syndrome. Lokhu kubizwa nge-adrenal nodular hyperplasia.

  • I-Familial Cushing syndrome. Ngokungavamile, abantu bafa baze bathole izigwaca kwenye noma ngaphezulu kwe-endocrine glands zabo, ezingamagland azenza ama-hormone. Uma lezi zigwaca zenze i-ACTH noma i-cortisol, i-Cushing syndrome ingahle ibe khona.

Ama-adrenal gland tumors noma isifo. Izinkinga nge-adrenal glands zingabangela ukuba zenze i-cortisol eningi. Okuvamile kakhulu yisigwaca esingxenyeni yangaphandle ye-adrenal gland ebizwa nge-adrenal adenoma. Lezi zigwaca azilona umdlavuza, futhi ezimbalwa kuphela ezenza i-cortisol eningi.

Izigwaca zomdlavuza ezingxenyeni zangaphandle ze-adrenal glands, ezibizwa nge-adrenocortical carcinoma, zimbalwa. Kodwa zingenza i-cortisol futhi zibangele i-Cushing syndrome. Ngezinye izikhathi, izicucu eziningi ezenza i-cortisol zingakhulayo e-adrenal glands futhi zibangele i-Cushing syndrome. Lokhu kubizwa nge-adrenal nodular hyperplasia.

Izingqinamba

Uma ingelaphiwa, i-Cushing syndrome ingabangela izinkinga, okuhlanganisa:

  • Ukwehluleka kwama-bone, okubizwa nangokuthi i-osteoporosis, okungabangela ukweqa kwama-bone.
  • I-type 2 diabetes.
  • Ukutheleleka okukhulu noma okuningi.
  • Ukwehluleka komthamo womzimba namandla.
Ukuxilongwa

Ukusebenzisa imithi ye-glucocorticoid kuyindlela evamile kakhulu yokuthola i-Cushing syndrome. Umhlinzeki wakho wezempilo angabuka yonke imithi yakho—amaphilisi, ukugonywa, amakhilimu kanye ne-inhalers—ukubona ukuthi usebenzisa yini imithi engabangela i-syndrome. Uma kunjalo, ngeke udinga ezinye izivivinyo.

Uma i-Cushing syndrome ibangelwa umzimba ukwenza i-cortisol eningi kakhulu, kungaba nzima ukuyihluza. Lokho kungenxa yokuthi ezinye izifo zinezimpawu ezifanayo. Ukuhluza i-Cushing syndrome kungaba inqubo ende futhi eyingxaki. Kuzodingeka ubone udokotela oqeqeshwe ezifweni ze-hormonal, obizwa ngokuthi yi-endocrinologist.

U-endocrinologist cishe uzokwenza ukuhlolwa komzimba futhi abuke izimpawu ze-Cushing syndrome, njengobuso obukhulu, i-hump emuva komhlane, kanye nokukhahlela okulula, oluqhekekile olunezindawo ezolukile.

Uma awuzange usebenzise imithi ye-glucocorticoid, lezi zivivinyo zingasiza ekutholeni imbangela ye-Cushing syndrome:

  • Uvivinyo lwesisu negazi. Lezi zivivinyo zilinganisa amazinga e-hormone futhi zibonisa ukuthi umzimba uyakwenza yini i-cortisol eningi kakhulu. Ngovivinyo lwesisu, ungacelwa ukuthi uqoqe isisu lakho phakathi namahora angu-24. I-Cortisol, i-ACTH nezinye i-hormones zilinganiswa emasampulini esisu negazi.

Umhlinzeki wakho wezempilo angase futhi akukhuthaze ezinye izivivinyo. Lezi zivivinyo zilinganisa amazinga e-cortisol ngaphambi nangemva kokusebenzisa imithi ye-hormone ukuqalisa noma ukuvimba i-cortisol.

  • Uvivinyo lwesaliva. Amazinga e-cortisol avame ukuphakama nokwehla phakathi nosuku. Kubantu abangenayo i-Cushing syndrome, i-cortisol iyancipha kusihlwa. Ngokubuka amazinga e-cortisol kusuka esampulini elincane lesaliva elikhokhwe ebusuku, iqembu lezokunakekelwa kwezempilo lingabona ukuthi amazinga e-cortisol aphakeme kakhulu yini.
  • Uvivinyo lwezithombe. I-CT noma i-MRI scans zingathatha izithombe ze-pituitary kanye ne-adrenal glands ukubona ukuthi kukhona yini okuvela, njengezinhlungu.
  • Ukukhipha igazi kwe-inferior petrosal sinus. Lovivinyo lungasiza ekuqondeni ukuthi i-Cushing syndrome ibangelwa yini yi-ACTH-producing pituitary adenoma, noma i-ACTH-producing tumor kwenye inhlangano. Ngovivinyo, amasampuli egazi athathwa ezinhlanzi ezicisha i-pituitary gland, ebizwa ngokuthi yi-inferior petrosal sinuses.

Phakathi novivinyo, unikezwa imithi nge-vein ukukusiza uhlale uzolile futhi ukhululeke. Umpompi omncane ubekwa endaweni yakho yomhlane noma yentamo futhi ugcina e-inferior petrosal sinuses ukuqoqa isampula legazi. Esinye isampula legazi sithathwa engalweni yakho. Ube usunikezwa imithi ebangela i-tumor ukwenza i-ACTH eningi, futhi amasampuli egazi athathwa futhi ezindaweni ezifanayo. Amazinga e-ACTH abeyelulwa phakathi kwezindawo ezimbili zesampula.

Uma amazinga e-ACTH aphakeme esampulini lesinus, inkinga ivela e-pituitary. Uma amazinga e-ACTH afana phakathi kwesisu kanye nengalo, inkinga ingaphandle kwe-pituitary gland.

Uvivinyo lwesisu negazi. Lezi zivivinyo zilinganisa amazinga e-hormone futhi zibonisa ukuthi umzimba uyakwenza yini i-cortisol eningi kakhulu. Ngovivinyo lwesisu, ungacelwa ukuthi uqoqe isisu lakho phakathi namahora angu-24. I-Cortisol, i-ACTH nezinye i-hormones zilinganiswa emasampulini esisu negazi.

Umhlinzeki wakho wezempilo angase futhi akukhuthaze ezinye izivivinyo. Lezi zivivinyo zilinganisa amazinga e-cortisol ngaphambi nangemva kokusebenzisa imithi ye-hormone ukuqalisa noma ukuvimba i-cortisol.

Ukukhipha igazi kwe-inferior petrosal sinus. Lovivinyo lungasiza ekuqondeni ukuthi i-Cushing syndrome ibangelwa yini yi-ACTH-producing pituitary adenoma, noma i-ACTH-producing tumor kwenye inhlangano. Ngovivinyo, amasampuli egazi athathwa ezinhlanzi ezicisha i-pituitary gland, ebizwa ngokuthi yi-inferior petrosal sinuses.

Phakathi novivinyo, unikezwa imithi nge-vein ukukusiza uhlale uzolile futhi ukhululeke. Umpompi omncane ubekwa endaweni yakho yomhlane noma yentamo futhi ugcina e-inferior petrosal sinuses ukuqoqa isampula legazi. Esinye isampula legazi sithathwa engalweni yakho. Ube usunikezwa imithi ebangela i-tumor ukwenza i-ACTH eningi, futhi amasampuli egazi athathwa futhi ezindaweni ezifanayo. Amazinga e-ACTH abeyelulwa phakathi kwezindawo ezimbili zesampula.

Uma amazinga e-ACTH aphakeme esampulini lesinus, inkinga ivela e-pituitary. Uma amazinga e-ACTH afana phakathi kwesisu kanye nengalo, inkinga ingaphandle kwe-pituitary gland.

Ukwelashwa

Ukwelapha i-Cushing syndrome kuqondiswe ekuthuthukiseni inani le-cortisol emzimbeni. Ukwelapha okuhle kunawe kuncike esizathwini se-syndrome. Izindlela zihlanganisa:

Uma i-Cushing syndrome ibangelwa ukusebenzisa imithi ye-glucocorticoid isikhathi eside, umhlinzeki wakho wezempilo angase akwazi ukulawula izimpawu zakho ngokunciphisa imithi oyithathayo. Lokhu kwenziwa ngokucophelela ngokuhamba kwesikhathi, kuyilapho usaphatha isimo osithathisayo. Unganciphisi umthamo wemithi ye-glucocorticoid noma uyiyeke wedwa. Yenza kanjalo kuphela ngosizo lomhlinzeki wakho wezempilo.

Ukuyeka le mithi ngokushesha kakhulu kungabangela ukuba ube ne-cortisol encane emzimbeni wakho. Ukuyeka imithi kancane kancane kuvumela umzimba wakho ukuba wenze inani le-cortisol elihle.

Uma i-Cushing syndrome ibangelwa yi-tumor, umhlinzeki wakho wezempilo angase akukhuthaze ukuba ususe i-tumor ngokuthatha. I-tumors ye-Pituitary ivame ukususwa yi-neurosurgeon, engase iyenze lokhu ngaphandle kwesango lakho. I-tumors evelisa i-ACTH ezingxenyeni zomzimba zingasuswa ngokuthatha okuvamile noma ngokusebenzisa izindlela ezingekho ezinzima ngokusebenzisa ukusoka okuncane.

Uma i-tumor evelisa i-ACTH ingatholakali, noma uma ingasuswa ngokuphelele futhi i-Cushing syndrome iqhubeka, umhlinzeki wakho wezempilo angase akukhuthaze ukuba ususe ama-adrenal glands. Lokhu kubizwa ngokuthi yi-bilateral adrenalectomy. Le nqubo imisa ngokushesha umzimba ukuba ungenzi i-cortisol eningi. Ngemva kokuba ama-adrenal glands omabili esusiwe, kungase kudingeke uthathe imithi ukuba ubeke indawo ye-cortisol ne-hormone enye ye-adrenal ebizwa ngokuthi yi-aldosterone ukuphila kwakho konke.

Ama-adrenal gland tumors angasuswa ngokusebenzisa ukusoka ezingxenyeni zomzimba noma emuva. Ngokuvamile, ama-adrenal gland tumors angekho umdlavuza angasuswa ngendlela engathinti kakhulu.

Ngemva kokuthatha i-Cushing syndrome, umzimba wakho ngeke wenze i-ACTH eyanele. Kuzodingeka uthathe imithi yokubeka indawo ye-cortisol ukunika umzimba wakho inani elifanele le-cortisol. Ngezinye izikhathi, umzimba wakho uqala ukwenza i-cortisol eyanele, futhi umhlinzeki wakho wezempilo anganciphisa imithi yokubeka indawo. I-endocrinologist yakho ingasebenzisa ukuhlolwa kwegazi ukuze usize ukunquma ukuthi udinga imithi ye-cortisol nokuthi ingayeki nini.

Le nqubo ingathatha kusuka ezinyangeni eziyisithupha kuya kuminyaka noma ngaphezulu. Ngezinye izikhathi, abantu abane-Cushing syndrome badinga imithi yokubeka indawo impilo yabo yonke.

Uma udokotela angakwazi ukususa ngokuphelele i-pituitary tumor, ukwelashwa ngomgogodla kungase kudingeke kanye nokuthathwa. Umgogodla ungase futhi usetshenziswe kubantu abangakwazi ukuthathwa.

Umgogodla unganikwa ngezilinganiso ezincane ngamasontwana ayisithupha, noma ngesilinganiso esiphezulu esisodwa somgogodla. Kuzo zombili izimo, umhlinzeki wakho wezempilo angahlela inqubo yakho ngendlela eunciphisa ukukhishwa komgogodla kwezinye izicubu.

Imithi ingasetshenziswa ukulawula amazinga e-cortisol uma ukuthathwa nomgogodla kungasebenzi noma akuyona inketho. Imithi ingase futhi isetshenziswe ngaphambi kokuthathwa kubantu abagulayo kakhulu nge-Cushing syndrome. Lokhu kungathuthukisa izimpawu zesifo futhi kunciphisa ingozi yokuthathwa. Ukwelashwa kwezokwelapha kwe-Cushing syndrome akuyona impumuzo futhi kungase kungathuthukisi ngokuphelele zonke izimpawu ze-cortisol eningi.

Imithi yokulawula ukukhiqizwa kwe-cortisol e-adrenal gland ihlanganisa i-ketoconazole, i-osilodrostat (Isturisa), i-mitotane (Lysodren), i-levoketoconazole (Recorlev), ne-metyrapone (Metopirone).

Mifepristone (Korlym, Mifeprex) ivunyelwe kubantu abane-Cushing syndrome abanesi-type 2 diabetes noma ushukela ophezulu. I-Mifepristone ayinciphisi inani le-cortisol umzimba owenza, kodwa ivala umphumela we-cortisol ezicubeni.

Pasireotide (Signifor) inikwa njengesiphuzo kabili ngosuku. Isebenza ngokunciphisa inani le-ACTH evela kwi-tumor, eunciphisa amazinga e-cortisol. Ezinye izidakamizwa ziyathuthukiswa.

Ngezinye izikhathi, i-tumor noma ukwelashwa kwayo kubangela i-pituitary noma i-adrenal gland ukuba yenze kancane kwezinye izinhlobo ze-hormone. Uma lokhu kwenzeka, umhlinzeki wakho wezempilo angakuncoma ukufakwa kwe-hormone.

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