Ukuba uye watshelwa ukuba unesifo se-endocrine se-neoplasia (MEN), ngoko uyazi ukuba kukho ukuvavanya ngokubanzi kunye nokunyangwa kwimeko engavumelekanga.
Inkcazo ye-MEN
Ininzi ye-endocrine neoplasia (i-MEN) isifo sezonyango esichazwa ngokuba ne-tumor i-organic endogine ngexesha. Kukho iintlobo ezahlukeneyo zezicubu ze-endocrine eziyaziwayo ukuba zenzeke kunye, kwaye nganye yeepateni zikwahlulwe njengenye yeendlela ezahlukeneyo ezihlukeneyo (MEN).
Iimvumi eziphuhliswa njengenxalenye yesifo ngasinye seMEN zingabonakalisa okanye zibi. Izibilini ze-Benign ziziimpawu ezizimeleyo ezingabangela iimpawu zezokwelapha kodwa zikhule ngokukhawuleza, zingabonakali kwezinye iindawo zomzimba kwaye azibulali. Ngakolunye uhlangothi, izicubu ezinomdla, ezinomdlavuza ongasakhula ngokukhawuleza, zingasasazeka kwezinye iindawo zomzimba, kwaye ziyakwazi nokuba zibulale xa zishiywa zingakhange zilahlwe.
Syndromes
Kukho ezintathu i-syndromes ezibhekwa njengezona zixhaphakileyo ze-MEN syndromes. Isifo ngasinye sibangelwa yinto engavamile yokuzalwa, oku kuthetha ukuba ukudibanisa i-tumor isebenza kwiintsapho njengelifa. Ama-syndromes e-MEN abizwa ngokuthi MEN 1, MEN 2A, kunye ne-MEN 2B.
ABANTU 1: Abantu abaxilongwe abane-MEN 1 baneemathumba zesigwebe, i-pathyity gland, ne-pancreas. Ngokuqhelekileyo, ezi zicubu zinobungozi, nangona kungenakwenzeka ukuba zibe zibi.
AMANZI 1 Izibonakaliso: Izimpawu ze-MEN 1 zingaqala ngexesha lobuncinci okanye ngexesha lomdala.
Iimpawu ngokwazo ziyahluka kuba izibilini zibandakanya izitho ze-endocrine ezingakwazi ukuvelisa uluhlu lweempembelelo emzimbeni. Ngamanye amathumba adala utshintsho olungaqhelekanga oluhambelana nokugqithiswa kwe-hormonal.
- Amathumba omzimba abangela intloko , ukunyuka kwexesha, ukungabikho komntwana, ukuvelisa ubisi lwebele kunye nokugqithisa kwamathambo.
- Amathumba asePancreatic enza intlungu yesisu, ukuhlanza, isifo sohudo kunye nezilonda.
- Izidumbu ze-Parathyroid zibangele amanqanaba aphezulu e-calcium, enokuthi ivelise amatye eentso njengesiqalo sokuqala esibonakalayo.
ABANTU 2A: Abantu abane-MEN 2 baneesifo se-thyroid, i-adrenal gland tumors, kunye ne-tumors.
AMANZI 2A Iimpawu: Iimpawu zeMEN 2A ziqala xa zikhulile; ngokuqhelekileyo xa umntu ehlala kwi-30s. Njengayezinye i-MEN syndromes, iimpawu zibangelwa ukugqithiseleka kwezicubu ze-endocrine.
- Iimvumba zeTyroid ziqhelekileyo zizidumbu zokuqala ezibonakalayo kwi-MEN 2A, kwaye zinokubangela ukuvuvukala okanye uxinzelelo kwingingqi yentamo, kwaye ziba zibi.
- I-Adrenal gland tumors, ebizwa ngokuba yi- pheochromocytoma , ibandakanya ngokuthe ngqo i-adrenal gland ebizwa ngokuba yi-adrenal medulla. I-Pheochromocytoma inokubangela uxinzelelo lwegazi oluphezulu, izinga lentliziyo ngokukhawuleza, nokukhupha.
- Izidumbu ze-Parathyroid zingabangela amanqanaba aphezulu e-calcium, kwaye iimpawu zingabandakanywa ukoma okweqile kwaye ukuvutha rhoqo.
AMANZI 2B: Le nto iyona eqhelekileyo kwezi ndlela ezinqabileyo zesisu kwaye ibonakala ngethambo le-thyroid, i-adrenal gland tumor, i-neuromas (i-cysts) emlonyeni kunye neenkqubo zokutya, ukungaqhelekanga kwesakhiwo samathambo, kunye nobude obuqhelekileyo kunye nobukhulu besikhumba obuchazwe njenge-marfanoid Iimbonakalo.
AMANZI 2B Iimpawu: Iimpawu zingabalwa ebuntwaneni, ngokuphindaphindiweyo ngaphambi kweminyaka eyi-10. Uphawu olubonakalisa kakhulu lwe-MEN 2B lubude obubonakalayo. I-Neuromas ibonakala njengokukhula kunye nasemlonyeni, kwaye abantu abane-MEN 2B banokuba neengxaki zesisu kunye neentsholongwane, ngaphezu kweempawu zomdlavuza we- thyroid kunye ne-pheochromocytoma.
Ukuxilongwa
Ugqirha wakho uya kuba nexhala lokuba unokuba ne-MEN syndrome ngokusekelwe kumzekelo wesifo se-endocrine esingaphezu kwesinye, kunye nomlando wentsapho yakho. Awudingi ukuba zonke izibonda zesayine enye ye-MEN syndromes ukuba ugqirha wakho aqwalasele i-MEN. Ukuba unesifo esingaphezulu kwesinye okanye isicatshulwa, okanye nokuba unesifo esisodwa se-endocrine edibene ne-MEN, ngoko ugqirha wakho unokukuvavanya ezinye iimvumi ngaphambi kokuba abe nempawu.
Ngokufanayo, imbali yentsapho ayikho imfuneko yokuxilongwa kweMEN, kuba umntu unokuba ngowokuqala kwintsapho ukuba abe nesifo. Izaziso ezithile ezenza i-MEN zichongwe, kwaye ukuhlolwa kofuzo kungabakho ukhetho ekuqinisekiseni ukuxilongwa.
Kukho iqondo lokuhlukahluka nge-syndrome nganye ye-MEN-unokuba no-MEN ngisho nokuba awuhambisani kakuhle neyodwa yamaphethini afanayo.
Unyango
Unyango lwazo zonke i-MEN syndromes lixhomekeke kwimibandela embalwa eyahlukileyo. Akuyena wonke umntu ofunyaniswa ukuba ene-MEN 1, MEN 2A, okanye uMEN 2B ufumana isifundo esifanayo esifanelekileyo sesifo. Ngokuqhelekileyo, unyango lujoliswe kwiinjongo ezintathu eziphambili, eziquka ukunciphisa iimpawu, ukufumana iintsholongwane kwangaphambili, nokukhusela imiphumo yezifo ezibi.
Ukuba unempawu ezibangelwa zizitenxo ze-hormone, mhlawumbi uya kufuneka unyango lwezo mpawu. Izifo ze-Endocrine zihlala ziguquguquka ngokuhamba kwexesha, ngenxa yokuba izitho ze-endocrine zisebenza ngokusekelwe kwinqanaba elincomekileyo leempendulo. Oku kuthetha ukuba i-hormone ebenzileyo ingagqiba ukukhupha okanye ukuvuselela ukuveliswa kwamanye amahomoni emzimbeni. Ngoko ke, nangona ufumana unyango lwe-hormone okwesikhashana, ungadinga unyango oluhlukile okanye utshintsho lomthamo wakho kwixesha elizayo.
Eminye imiba ebalulekileyo yonyango lwe-MEN iquka ukuhlolisiswa ngokucophelela nokubeka iliso ukufumanisa izicubu ezintsha kwaye ubone naluphi na ukulimala ngokukhawuleza. Izidumbu ezibi zingadinga ukunyangwa ngokuhlinzwa okanye i-chemotherapy phambi kokuba zenze ezinye iingxaki zempilo.
ILizwi
Naluphina uhlobo lwesifo esinokuqhayisa. Kwaye kunokuba kunomdla ongaphezu kweyodwa. Ukuba uye watshelwa ukuba unayo okanye unokuba neoplasia ye-endocrine ephezulu, mhlawumbi unomdla kakhulu malunga nokuba yimpawu ezingaphila ngokuzayo kwaye ingaba impilo yakho yonke iyingozi.
Inyaniso yokuba i-MEN syndromes iyabonwa kwaye ikwahlula yenza imeko yakho iboneke ngakumbi kunokuba ibonakala ngathi. Nangona kunjalo ukuba ezi zizinto ezinqabileyo zi-syndromes, ziye zachazwa ngokuchanekileyo kwaye zikho iindlela ezichanekileyo zokulawula ezi zifo. Nangona ngokuqinisekileyo uya kufuneka ulandelelwe unyango oluqhubekayo, kukho iindlela ezifanelekileyo zokulawula imeko yakho kwaye unokuqiniseka ngokuqinisekileyo ukuba unonophelo olusondeleyo lwezempilo, unokufumana impembelelo enempilo.
> Umthombo:
> Wasserman JD, Tomlinson GE, Druker H, et al. Iimpawu ezininzi ze-Endocrine Neoplasia kunye ne-Hyperparathyroid-I-Jaw Tumor Syndromes: Iinkqubo zeeNklinikhi, i-Genetics, kunye neengcebiso kwi-Childhood. Can Cancer Res . 2017 Julayi 1; 23 (13): e123-e132.