Masifunde ngezinye iingxaki ngenkqubo ye-glandular yomzimba wakho? - I-Multiple Endocrine Neoplasia (MEN)

Umzimba wethu ufana nomatshini omangalisayo, akunjalo? Ukuze lo matshini usebenze ngokufanelekileyo, inxalenye nganye kufuneka isebenzisane kwaye ihambelane ngokufanelekileyo. Kukho inkqubo ebaluleke kakhulu emzimbeni wethu, ebizwa ngokuba yinkqubo ye-endocrine . Amadlala ale nkqubo avelisa iihomoni ezilawula imisebenzi emininzi emzimbeni wethu. Nangona kunjalo, ngamanye amaxesha le nkqubo inokungahambi kakuhle. Namhlanje siza kuthetha ngemeko engaqhelekanga kodwa ebaluleke kakhulu ekufuneka siyiqaphele. Le yiMultiple Endocrine Neoplasia , ekwaziwa ngokuba yi-`(MEN)` ngamafutshane.

Ngoko ke yintoni le nkqubo ye-endocrine?

Ngamafutshane, inkqubo yethu ye-endocrine yinethiwekhi yamadlala kunye namalungu omzimba emzimbeni wethu. La ngawo avelisa iikhemikhali ezibizwa ngokuba zii-hormones aze azikhuphele egazini. Cinga ngala ma-hormones njengezithunywa ezincinci ezithwala imiyalezo ngaphakathi emizimbeni yethu. Le miyalezo ixelela amalungu ethu, ulusu, kunye nemisipha into ekufuneka siyenze kunye nexesha ekufuneka siyenze ngalo.

Kukho iindlala ezininzi eziphambili kunye namalungu omzimba ayinxalenye yenkqubo yethu ye-endocrine:

• I-Hypothalamus: Le ikwisiseko sobuchopho bethu. Ilawula izinto ezininzi kwinkqubo ye-endocrine.
• I-pituitary gland: Le gland incinci, edibene ne-hypothalamus, ivelisa iihomoni ezilawula ezinye iihomoni ezininzi, kuquka i-thyroid gland, i-adrenal glands, ii-ovari, kunye namasende.
• I-thyroid: Le gland imile okwebhabhathane engaphambili entanyeni ilawula imetabolism yomzimba wethu, yindlela esisebenzisa ngayo amandla avela ekutyeni esikutyayo.
• Iindlala zeParathyroid: Ezi ndlala zine zincinci, ezihlala zikufutshane nendlala ye-thyroid, zilawula amanqanaba e-calcium kunye ne-phosphorus emzimbeni wethu.
• Iindlala ze-adrenal: Ezi ndlala zimbini, ezikwindawo engaphezulu kwezintso, zilawula imetabolism, uxinzelelo lwegazi, uphuhliso lwesondo, kunye nempendulo yoxinzelelo.
• I-pineal gland: Le gland ivelisa i-hormone i-melatonin kwaye ilawula umjikelo wethu wokulala.
• I-Pancreas: Kulapho iveliswa khona i-insulin. Ibaluleke kakhulu kwi-metabolism kunye nolawulo lweswekile egazini. Ikwayinxalenye yenkqubo yethu yokugaya ukutya.
• Amaqanda: Kulapho kuveliswa khona iihomoni zesini zabasetyhini i-estrogen, i-progesterone, kunye ne-testosterone.
• Ama-Testes: Ngelixa kuveliswa isidoda emadodeni, i-hormone ye-testosterone nayo iveliswa apha.

Ngoko ke yintoni iMultiple Endocrine Neoplasia (MEN)?

I-Multiple Endocrine Neoplasia (MEN) yimeko engaqhelekanga yemfuza apho iithumba zikhula khona kwiindlala kunye nezicubu ezingaphezulu kwesinye zenkqubo ye-endocrine, esixoxe ngazo ngaphambili. Ngamanye amaxesha ezi thumba zinokuba ngumhlaza.

Kukho iintlobo ezimbini eziphambili zale meko `(MEN)`:

1. I-Multiple endocrine neoplasia type 1 (MEN type 1): Le yimeko apho iithumba ezininzi zikhula khona kwiindlala ezahlukeneyo zenkqubo ye-endocrine. Oku kukwabizwa ngokuba yi-MEN-1 kunye ne-Wermer's syndrome.

2. I-Multiple endocrine neoplasia type 2 (MEN type 2): Le yimeko yomhlaza wemfuza echaphazela amadlala aliqela. Abantu abane-MEN2 bahlakulela uhlobo lomhlaza we-thyroid olubizwa ngokuba yi-medullary thyroid cancer (MTC) . Bakwanomngcipheko omkhulu wokufumana amathumba kwamanye amadlala e-endocrine system. Oku kukwabizwa ngokuba yi-MEN-2 kunye ne-Sipple syndrome.

Kutheni le meko `(YABANTU ABANEMISEBENZI)` isenzeka?

Zombini ezi ntlobo ze-`(MEN)` zibangelwa lutshintsho kwiijini zethu, oko kukuthi, utshintsho . Khawucinge ukuba imizimba yethu inento efana nencwadi yemiyalelo, oko kukuthi, iijini. Ukuba unobumba omnye kule ncwadi yemiyalelo akachanekanga, oko kukuthi, ukuba ijini iyatshintsha, eminye yemisebenzi yomzimba inokuphazamiseka.

• (Uhlobo 1 lwe-MEN) lubangelwa kukuguquka kwezakhi zofuzo ze-`MEN1` . Le gene ye-`MEN1` yi -gene ethintela ithumba . Oko kukuthi, i-gene enceda ukulawula ukwahlukana kweeseli kunye nokuthintela ukwakheka kweethumba. Ukuba le gene ayisebenzi kakuhle, ezinye iiseli zinokukhula zingalawuleki kwaye zenze iithumba.
• (Uhlobo lwe-MEN 2) lubangelwa kukuguquka kwejini ye-`RET` . Le jini ye-`RET` yijini enxulunyaniswa nophuhliso lomhlaza. Xa le jini itshintshatshintsha, iiseli kwamanye amalungu kunye namadlala zikhula ngokungalawulekiyo kwaye zenze amathumba.

Olu tshintsho lwemfuza lunokufunyanwa kubazali okanye lunokwenzeka ngokungacwangciswanga ngexesha lokukhula komntwana ongekazalwa. Ukuba omnye umzali une-MEN, kukho amathuba angama-50% okuba umntwana akhule nalo.

Masifunde okungakumbi malunga nohlobo `(MEN)` (`MEN uhlobo 1`)

Abantu abane-MEN type 1 bahlakulela iithumba kwiindlala ezininzi zenkqubo ye-endocrine. Iindawo ezichaphazeleka kakhulu zezi:

• Iindlala zeParathyroid: Le yeyona ixhaphakileyo.
• Indlela yesisu ebangela i-pancreatic tract: Oku kuthetha ukuba iithumba zinokwakheka kwi-pancreas, esiswini, nakwezinye iindawo zenkqubo yokugaya ukutya, njenge-duodenum.
• Indawo yolwimi lamathe.

Uninzi lwexesha, amaqhuma akhula ngohlobo lwe-MEN 1 awabi ngumhlaza (angabi yingozi) . Nangona kunjalo, amanye amaqhuma anokuba ngumhlaza (anobungozi).Ingasasazeka (i-metastasize) nakwezinye iindawo zomzimba. Amadlala aneethumba adla ngokukhupha iihomoni ezininzi egazini. Yiyo le nto ibangela iimpawu ezahlukeneyo kunye neengxaki zempilo.

Oogqirha bafumanise iintlobo ezingaphezu kwama-20 zee-endocrine tumors kunye nee-non-endocrine tumors kubantu abane-MEN type 1. Kukho ezinye iintlobo zee-tumors ezingaqhelekanga, ezifana nezi:

• Ii-neuroendocrine tumors ezikhula kwi-thymus (i-lymph node esifubeni) kunye ne-bronchi (iindlela zomoya emiphungeni).
• Ii-tumor ze-Adrenocortical zenzeka kumaleko wangaphandle wee-adrenal glands.
• Amaqhuma anamafutha (iiLipomas) aqala phantsi kolusu.
• Umhlaza webele.
• Iithumba ezifana ne -meningiomas kunye ne -ependymomas ezinokwenzeka engqondweni okanye emgogodleni.

Zithini iimpawu ze-`(MEN type 1)`?

Iimpawu ze-MEN type 1 zinokwahluka kakhulu kumntu nomntu. Kuxhomekeke ekubeni zeziphi ii-gland ezichaphazelekileyo kunye nokuba zivelisa i-hormone engakanani. Abanye abantu basenokungabi nazo iimpawu konke konke, abanye banokuba neempawu ezingephi, kwaye abanye banokuba neempawu ezinzima, ezisongela ubomi. Iimpawu zinokwahluka kumntu nomntu, nokuba kusapho olunye, nokuba phakathi kwamawele.

Makhe sijonge ezinye zezona ntlobo ziphambili zamaqhuqhuva kunye neempawu ezinxulumene nawo:

• Ngenxa yeengxaki ze-parathyroid glands (hyperparathyroidism):

Ama-90% abantu abane-`(MEN type 1)` bakhula nale meko xa beneminyaka engama-50 ubudala. Kule meko, amadlala e-parathyroid asebenza kakhulu.

• Iimpawu ezibuthathaka: iintlungu zamalungu, ubuthathaka bemisipha, ukudinwa, ukudakumba, ubunzima bokugxila, ukuphelelwa ngumdla wokutya.
• Iimpawu eziqatha: isicaphucaphu, ukuhlanza, ukudideka, ukulibala, ukoma kakhulu kunye nokuchama rhoqo, ukuqhina, iintlungu zamathambo.

• Ngenxa yeethumba kwi-pancreas nakwinxalenye yokuqala yamathumbu amancinci (i-Gastrinomas):

Malunga nama-40% abantu abadala abane-MEN type 1 bahlakulela iithumba ezibizwa ngokuba yi-gastrinomas. Ezi zivelisa ihomoni ebizwa ngokuba yi-gastrin, ebangela ukuba isisu sivelise i-asidi eninzi kakhulu.

• Iimpawu: Intlungu yesisu, urhudo, isitshiso sentliziyo (i-acid reflux), izilonda zesisu (izilonda zesisu).
• Esinye isifo esibonakala kolu didi yi -insulinoma . Esi sifo sivelisa i-insulin, enokubangela iswekile ephantsi egazini (hypoglycemia).
• Iimpawu: ukudideka, ukungcangcazela, ukubila, indlala egqithisileyo, ukungazinzi, ukubetha kwentliziyo ngokukhawuleza, utshintsho lwembono okwethutyana.

• Ngenxa yeethumba zepituitary (iiprolactinomas):

Malunga nama-25% abantu abane-MEN type 1 bahlakulela iithumba zepituitary. Ezona zixhaphakileyo zii -prolactinomas, ezivelisa i-hormone prolactin .

• Iimpawu kubafazi: ukungaqheleki okanye ukuyeka ukuya exesheni (i-amenorrhea), ubunzima bokukhulelwa abantwana, ukuphuma kobisi oluvela kwiingono xa bengakhulelwanga okanye bengancancisi (i-galactorrhea), ukuncipha komnqweno wesondo.
• Iimpawu kumadoda: Ukuncipha komnqweno wesondo ngenxa yokwehla kwamanqanaba e-testosterone, ukungasebenzi kakuhle kwe-erectile (ED), kunye nobunzima bokukhulelwa abantwana.
• Ukuba iqhuma likhulu: intloko ebuhlungu, isicaphucaphu/ukuhlanza, utshintsho lombono (ukubona kabini, ukuncipha kokubona ecaleni).

Kubalulekile: Asinguye wonke umntu oza kuba neempawu ezifanayo. Olu luhlu aluphelelanga. Kukho ezinye iintlobo ezininzi zamaqhuqhuva anokuvela kwi-`(MEN type 1)`.

Masifunde nangohlobo `(AMADODA)` (`AMADODA uhlobo 2`)

Wonke umntu one-MEN type 2 uba nohlobo lomhlaza we-thyroid olubizwa ngokuba yi-medullary thyroid cancer (MTC) . Olu hlobo lomhlaza luvela kuhlobo oluthile lweseli kwi-thyroid gland ebizwa ngokuba zii-C cells. Ezi seli zivelisa i-hormone ebizwa ngokuba yi-calcitonin. I-MTC inokusasazeka kwii-lymph nodes nakwezinye izitho zomzimba. Unyango oluphambili kukususwa kwe-thyroid gland ngotyando (thyroidectomy).

Kwakhona, abantu abane-`(MEN type 2)` banokufumana enye okanye zombini ezi meko zilandelayo:

• I-Pheochromocytoma: Le yithumba elingaqhelekanga elikhula embindini wesinye okanye zombini ii-adrenal glands (i-adrenal medulla). Uninzi lwexesha, ezi azinawo umhlaza. Malunga ne-10%-15% yamatyala anokuba ngumhlaza aze asasazeke.
• I-Hyperparathyroidism: Amanqanaba e-calcium egazini ayanda ngenxa yokuveliswa kakhulu kwe-parathyroid hormone (PTH) yi-parathyroid glands.

Zithini iimpawu ze-`(MEN type 2)`?

Iimpawu apha nazo zahlukile. Iimpawu zihlala zibangelwa ngamanqanaba aphezulu eehomoni eziveliswa ngumhlaza (MTC) kunye nezinye iithumba.

• Iimpawu zomhlaza we-thyroid obizwa ngokuba yi-medullary thyroid cancer (MTC):
• Iqhuma elingaphambili entanyeni, intlungu entanyeni, utshintsho lwelizwi (ukurhawuzelela), ukukhohlela, ubunzima bokuginya, ubunzima bokuphefumla.

• Iimpawu ezibangelwa yiPheochromocytoma: (zenzeka malunga nama-50% amatyala)
• Iimpawu zenzeka kuphela xa ezi thumba zivelisa iihomoni ezininzi kakhulu, ezifana ne-adrenaline. Ezinye iithumba azivelisi iihomoni kwaye zisenokungabonakalisi zimpawu.
• Iimpawu eziqhelekileyo (ezihlala zifika ziphinde zidlule): uxinzelelo lwegazi oluphezulu (uxinzelelo lwegazi oluphezulu), intloko ebuhlungu, ukubila okugqithisileyo ngaphandle kwesizathu, ukubetha kwentliziyo okukhawulezayo okanye okungahambelaniyo, ukungcangcazela.

• Iimpawu ezibangelwa yi-hyperparathyroidism:
• Njengakwi-`(uhlobo 1 lwe-MEN)`, iimpawu ezinje ngentlungu yamalungu, ubuthathaka bemisipha, ukudinwa, ukudakumba, ubunzima bokugxila, ukuphelelwa ngumdla wokutya, isicaphucaphu, ukuhlanza, ukoma kakhulu kunye nokuchama rhoqo, ukuqhina, kunye nentlungu yamathambo zinokubakho.

Ngubani onokuhlakulela le meko `(AMADODA)`? Ixhaphake kangakanani?

Le meko `(MEN)` inokuchaphazela amadoda nabafazi ngokulinganayo. Ubudala apho le meko `(MEN type 1)` ibonakala khona buyahluka kakhulu. Ifunyaniswe kubantwana abaneminyaka esi-8 ubudala kunye nabantu abadala abaneminyaka engama-80 ubudala.

`(AMADODA)` yimeko engaqhelekanga .

• `(Uhlobo 1 lwe-MEN)` luchaphazela umntu omnye kwabangama-30,000.
• `(Uhlobo lwe-MEN 2)` luchaphazela umntu omnye kubantu abangama-35,000.

Abanye abaphandi bakholelwa ukuba inani labantu abanezi meko linokuba phezulu ngenxa yokungafumani ngqwalasela okanye ukungazifumani ngokuchanekileyo.

Oogqirha bayixilonga njani imeko ethi `(MEN)`? (Ukuxilongwa)

Ukuchonga imeko ethi `(MEN)` kunokuba nzima kancinci, kuba ichaphazela amadlala ahlukeneyo.

• Ukuchongwa kwe-`(uhlobo lwe-MEN 1)`:

Umntu udla ngokufunyaniswa enohlobo lwe-MEN 1 ukuba unezibilini ezimbini kwezithathu eziphambili ze-endocrine ezikhankanyiweyo apha ngasentla (izibilini ze-parathyroid, izibilini ze-pituitary, kunye/okanye izibilini zendlela yokugaya ukutya), okanye ukuba unenye yezo zibilini kwaye ilungu losapho linohlobo lwe-MEN 1.

• Uvavanyo:
• Uvavanyo lwegazi: Jonga amanqanaba aphezulu eehomoni ezithile (umz. i-parathyroid hormone (PTH), i-calcium).
• Uvavanyo lokufota: Olu vavanyo lunokunceda ekufumaneni iindawo ezinamaqhuma, njenge-CT scan (i-computed tomography scan) okanye i-MRI (i-magnetic resonance imaging scan).
• Uvavanyo lwemfuza: Ujonga utshintsho kwimfuza ye-`MEN1`.

• Ukuchongwa kwe-`(uhlobo lwe-MEN 2)`:

Umntu ufunyaniswa enohlobo lwe-MEN 2 ukuba unomhlaza we-thyroid obizwa ngokuba yi-medullary thyroid cancer (MTC) kunye ne-pheochromocytoma kunye/okanye i-hyperplasia okanye i-adenoma yee-parathyroid glands.

• Uvavanyo:
• Uvavanyo lwegazi: Jonga amanqanaba eehomoni afana ne-calcitonin (ye-MTC), i-parathyroid hormone (PTH), kunye ne-catecholamines (ye-pheochromocytoma).
• Uvavanyo lokufota: `i-CT scan` okanye `i-MRI scan` ziyasetyenziswa.
• Uvavanyo lwemfuza: Ujonga utshintsho kwimfuza ye-'RET'.

Ziziphi iindlela zonyango ze-`(MEN)`?

Unyango lwe-MEN luxhomekeke ngokupheleleyo ekubeni zeziphi iindlala ze-endocrine kunye namalungu omzimba achaphazelekileyo. Ngokuqhelekileyo lubandakanya iqela loogqirha abahlukeneyo, kuquka iingcali ze-endocrinologists , oogqirha botyando , kunye neengcali ze-oncologists .

Kunokubakho iindlela ezahlukeneyo zonyango:

• Amayeza: Lawula iimpawu kwaye unciphise imiphumo yeehomoni ezigqithisileyo.
• Utyando: Susa iqhuma okanye yonke i-gland echaphazelekayo (umz., i-thyroid gland).
• Unyango lokutshintshwa kwamahomoni: Ukuba i-endocrine gland isusiwe ngotyando, amahomoni aveliswa yiyo anikwa ngaphandle.
• Unyango lomhlaza: Ukuba umhlaza usasazeke kwezinye iindawo (ezisasazeke kwi-metastases), unyango olufana ne -chemotherapy kunye nonyango lwe-radiation .

Ngenxa yokuba isigulana ngasinye `(AMADODA)` sahlukile, isicwangciso sonyango esisebenzela wonke umntu sahlukile. Musa ukoyika ukuthetha nogqirha wakho malunga neendlela zonyango ezikulungeleyo.

Ngaba `(AMADODA)` anganyangeka ngokupheleleyo? Ngaba angathintelwa?

Ngelishwa, okwangoku akukho nyango lwesifo `(MEN)`. Nangona kunjalo, sinokulawulwa . Oogqirha baphatha utshintsho kwi-gland nganye ngotyando okanye amayeza afanelekileyo ngelo xesha.

Akunakwenzeka ukuthintela uphuhliso lwe-`(MEN)`, kuba lubangelwa kukuguquka kwezakhi zofuzo. Ezi nguqu zezakhi zofuzo zinokufunyanwa kubazali okanye zenzeke ngokungacwangciswanga ngexesha lesigaba somntwana osandul’ ukuzalwa.

Nangona kunjalo, ukuba ilungu losapho elisondeleyo (umzali, umntakwenu okanye umntakwenu) lifunyaniswe line-MEN, kubalulekile ukuthetha nogqirha wakho malunga novavanyo lwemfuza lwe-MEN. Ukwenza njalo kunokunceda ekufumaneni amathumba kwangethuba ukuba unale meko.

Kwenzeka ntoni xa uhlala ne-`(AMADODA)`? (Ingxelo yokuxela kwangaphambili)

Ukubikezela kwe-MEN kuxhomekeke kwizinto ezininzi:

• Indlela ekwafunyanwa ngayo ngokukhawuleza `(MEN)`.
• Zeziphi iindlala ze-endocrine ezichaphazelekileyo?
• Nokuba iqhuma linomhlaza okanye akunjalo.
• Uhlobo lonyango olusetyenzisiweyo.
• Kubizwa ngokuba sisisu esinomhlaza esisasazeke kwezinye iindawo zomzimba (esinomhlaza).

Ukuba ufunyaniswe une-MEN, ugqirha wakho angakunika umbono ongcono malunga nonyango lwakho kunye nendlela eya phambili.

Ixesha lokubona ugqirha kunye nemibuzo ebalulekileyo ekufuneka uyibuze

Ukuba ufunyaniswe une-MEN, kuya kufuneka ubone ugqirha wakho rhoqo ukuze ajonge imeko yakho kwaye aqinisekise ukuba unyango lwakho lusebenza kakuhle.

Kwakhona, ukuba ilungu losapho olusondeleyo kusapho lwakho line-`(MEN)`, thetha nogqirha wakho malunga novavanyo lwemfuza, njengoko bekutshiwo ngaphambili.

Ukuba une-`(MEN)`, kunokuba luncedo ukubuza ugqirha wakho imibuzo efana nale:

• Luhlobo luni lwe-`(MEN)` endinalo?
• (MEN) iwuchaphazela njani umzimba wam?
• Zeziphi iimpawu ekufuneka ndizijonge?
• Ziziphi iindlela zonyango endinazo?
• Ziziphi iingenelo kunye neengxaki zeendlela ezahlukeneyo zonyango?
• Kuthatha ixesha elingakanani ukuba unyango luphumelele?
• Ngaba lonke usapho lwam kufuneka luvavanywe (AMADODA)?

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

I-multiple endocrine neoplasia (MEN) yimeko engaqhelekanga. Kwakhona, kuba AMADODA anokubangela iimpawu ezahlukeneyo kwaye imeko yawo wonke umntu ayifani, ngamanye amaxesha kunokuba nzima ukwazi ukuba unayo na.

Ukuba ufumanisa naziphi na iimpawu ezintsha ezikukhathazayo, okanye ukuba ubona naluphi na utshintsho emzimbeni wakho, eyona nto ingcono onokuyenza kukubona ugqirha.

Nangona ezinye iimeko ze-`(MEN)` zenzeka ngokungacwangciswanga, zinokuqhubeka nakwiintsapho. Ukuba omnye wezalamane zakho ezisondeleyo une-`(MEN)`, kubaluleke kakhulu ukufumana uvavanyo lwe-genetic ukuze ufumane ukuba usemngciphekweni na. Thetha nogqirha wakho ngayo nayiphi na imibuzo onayo malunga nomngcipheko wakho wokuba ne-`(MEN)`. Bakho ukukunceda. Musa ukoyika, ungoyiki, kwaye ungoyiki ukubuza imibuzo.

` I-neoplasia ye-endocrine emininzi, i-MEN, inkqubo ye-endocrine, iihomoni, iithumba, umhlaza, i-MEN uhlobo loku-1, i-MEN uhlobo lwesi-2, i-parathyroid, i-pituitary, i-pancreas, i-thyroid, i-MTC, i-pheochromocytoma, imeko yemfuza, iimpawu, ukuxilongwa, unyango