Ake sifunde ngezinye izinkinga ngohlelo lwezindlala lomzimba wakho? - I-Multiple Endocrine Neoplasia (MEN)

Umzimba wethu ufana nomshini omangalisayo, akunjalo? Ukuze lo mshini usebenze kahle, ingxenye ngayinye idinga ukusebenzisana futhi ixhumane kahle. Kukhona uhlelo olubaluleke kakhulu emzimbeni wethu, olubizwa ngokuthi uhlelo lwe-endocrine . Ama-gland akulolu hlelo akhiqiza ama-hormone alawula imisebenzi eminingi emzimbeni wethu. Kodwa-ke, ngezinye izikhathi lolu hlelo lungase luhambe kabi. Namuhla sizokhuluma ngesimo esingavamile kodwa esibaluleke kakhulu okufanele sisiqaphele. Leso yi -Multiple Endocrine Neoplasia , eyaziwa nangokuthi `(MEN)` ngamafuphi.

Ngakho-ke luyini lolu hlelo lwe-endocrine?

Kalula nje, uhlelo lwethu lwe-endocrine luyinethiwekhi yezindlala nezitho zomzimba emzimbeni wethu. Lezi yizo ezikhiqiza amakhemikhali abizwa ngokuthi ama-hormone bese ziwakhipha egazini. Cabanga ngala ma-hormone njengezithunywa ezincane ezithwala imiyalezo emizimbeni yethu. Le miyalezo itshela izitho zethu, isikhumba, nemisipha ukuthi senzeni nokuthi sikwenze nini.

Kunezindlala eziningana eziyinhloko nezitho zomzimba eziyingxenye yesistimu yethu ye-endocrine:

• I-Hypothalamus: Lokhu kutholakala phansi kobuchopho bethu. Kulawula izinto eziningi ohlelweni lwe-endocrine.
• Indlala ye-pituitary: Le ndlala encane, exhunywe kwi-hypothalamus, ikhiqiza ama-hormone alawula ezinye izindlala eziningi, okuhlanganisa indlala ye-thyroid, izindlala ze-adrenal, ama-ovari, nama-testicles.
• I-thyroid: Le ndlala emise okwebhabhathane engaphambili entanyeni ilawula ukusebenza komzimba wethu, okuyindlela esisebenzisa ngayo amandla avela ekudleni esikudlayo.
• Izindlala ze-Parathyroid: Lezi zindlala ezine ezincane, ezivame ukutholakala eduze kwendlala ye-thyroid, zilawula amazinga e-calcium ne-phosphorus emzimbeni wethu.
• Izindlala ze-adrenal: Lezi zindlala ezimbili, ezitholakala ngaphezu kwezinso, zilawula ukugaywa kokudla, umfutho wegazi, intuthuko yocansi, kanye nokusabela ekucindezelekeni.
• I-pineal gland: Le gland ikhiqiza i-hormone i-melatonin futhi ilawula umjikelezo wethu wokulala.
• I-Pancreas: Yilapho kukhiqizwa khona i-insulin. Ibaluleke kakhulu ekugayweni kwe-metabolism kanye nokulawula ushukela egazini. Iphinde ibe yingxenye yesistimu yethu yokugaya ukudla.
• Ama-ovary: Yilapho kukhiqizwa khona ama-hormone ocansi abesifazane i-estrogen, i-progesterone, ne-testosterone.
• Amasende: Ngesikhathi kukhiqizwa isidoda emadodeni, i-hormone i-testosterone nayo ikhiqizwa lapha.

Ngakho-ke iyini i-Multiple Endocrine Neoplasia (MEN)?

I-Multiple Endocrine Neoplasia (MEN) yisimo esingavamile sofuzo lapho izimila zikhula khona ezingxenyeni ezingaphezu kweyodwa zesistimu ye-endocrine, esixoxe ngayo ngaphambili. Ngezinye izikhathi lezi zimila zingaba umdlavuza.

Kunezinhlobo ezimbili eziyinhloko zalesi simo `(MEN)`:

1. I-Multiple endocrine neoplasia uhlobo 1 (MEN uhlobo 1): Lesi yisimo lapho kuvela khona izimila eziningi ezindlala ezahlukene zesistimu ye-endocrine. Lokhu kubizwa nangokuthi i-MEN-1 kanye ne-Wermer's syndrome.

2. I-Multiple endocrine neoplasia uhlobo 2 (MEN uhlobo 2): Lesi yisimo somdlavuza we-genetic esithinta izindlala eziningana. Abantu abane-MEN2 bahlakulela uhlobo lomdlavuza we-thyroid olubizwa ngokuthi i-medullary thyroid cancer (MTC) . Baphinde babe nengozi enkulu yokuthola izimila kwezinye izindlala ze-endocrine system. Lokhu kubizwa nangokuthi i-MEN-2 kanye ne-Sipple syndrome.

Kungani lesi simo `(MEN)` senzeka?

Zombili lezi zinhlobo ze-`(MEN)` zibangelwa izinguquko ezakhiweni zethu zofuzo , okungukuthi, izinguquko . Cabanga ukuthi imizimba yethu inento efana nencwadi yemiyalelo, okungukuthi, izakhi zofuzo. Uma ngisho nohlamvu olulodwa kule ncwadi yemiyalelo lungalungile, okungukuthi, uma izakhi zofuzo ziguquguquka, eminye yemisebenzi yomzimba ingaphazamiseka.

• (Uhlobo 1 lwe-MEN) lubangelwa ukuguquka kwezakhi zofuzo ze-`MEN1` . Le zakhi zofuzo ze-`MEN1` iyizakhi zofuzo ezicindezela isimila . Okusho ukuthi, izakhi zofuzo ezisiza ukulawula ukuhlukana kwamaseli nokuvimbela ukwakheka kwamathumba. Uma le zakhi zofuzo zingasebenzi kahle, amanye amaseli angakhula ngaphandle kokulawulwa futhi akhe amathumba.
• (Uhlobo lwe-MEN 2) lubangelwa ukuguquka kwezakhi zofuzo ze-`RET` . Lesi sakhi sofuzo se-`RET` siyisakhi sofuzo esihlotshaniswa nokukhula komdlavuza. Uma lesi sakhi sofuzo siguquguquka, amaseli kwezinye izitho nezindlala akhula ngokungalawuleki futhi akhe amathumba.

Lezi zinguquko zofuzo zingatholakala kubazali noma zingavela ngokungahleliwe ngesikhathi sokukhula kombungu. Uma umzali oyedwa ene-MEN, kunethuba elingama-50% lokuthi ingane izokhula.

Ake sifunde okwengeziwe ngohlobo `(MEN)` (`MEN uhlobo 1`)

Abantu abane-MEN type 1 bahlakulela amathumba ezindlala eziningana zesistimu ye-endocrine. Izindawo ezivame ukuthinteka yilezi:

• Izindlala ze-Parathyroid: Lokhu yikona okuvame kakhulu.
• Indlela yesisu: Lokhu kusho ukuthi izimila zingakheka kuma-pancreas, esiswini, nakwezinye izingxenye zesimiso sokugaya ukudla, njenge-duodenum.
• Indlala ye-pituitary.

Esikhathini esiningi, amaqhubu akhula ngohlobo lwe-MEN 1 awawona umdlavuza (angabi yingozi) . Kodwa-ke, amanye amaqhubu angaba umdlavuza (ayingozi).Kungasakazeka futhi (kusakazeke) kwezinye izingxenye zomzimba. Izindlala ezinezimila zivame ukukhulula ama-hormone amaningi egazini. Yilokhu okubangela izimpawu ezahlukahlukene nezinkinga zempilo.

Odokotela bathole izinhlobo ezingaphezu kuka-20 zezimila ze-endocrine kanye nezingezona eze-endocrine kubantu abane-MEN type 1. Kunezinye izinhlobo zezimila ezingavamile, njenge:

• Izimila ze-neuroendocrine ezikhula ku-thymus (i-lymph node esifubeni) kanye ne-bronchi (imigudu yomoya emaphashini).
• Izimila ze-adrenal cortex zakha ungqimba lwangaphandle lwezindlala ze-adrenal.
• Amaqhubu anamafutha (ama-Lipomas) akheka ngaphansi kwesikhumba.
• Umdlavuza webele.
• Izimila ezifana ne -meningiomas kanye ne-ependymomas ezingavela ebuchosheni noma emgogodleni.

Ziyini izimpawu ze-`(MEN type 1)`?

Izimpawu zohlobo lwe-MEN 1 zingahluka kakhulu kumuntu nomuntu. Kuya ngokuthi yiziphi izindlala ezithintekile nokuthi zikhiqiza ama-hormone angakanani. Abanye abantu bangase bangabi nazimpawu nhlobo, abanye bangase babe nezimpawu ezincane, kanti abanye bangase babe nezimpawu ezinzima, ezisongela ngisho nokuphila. Izimpawu zingahluka kumuntu nomuntu, ngisho nangaphakathi komndeni ofanayo, ngisho naphakathi kwamawele.

Ake sibheke ezinye zezinhlobo eziyinhloko zamaqhubu kanye nezimpawu zawo ezihambisana nawo:

• Ngenxa yezinkinga ngezindlala ze-parathyroid (i-hyperparathyroidism):

Abantu abangu-90% abane-`(MEN type 1)` baba nalesi simo lapho beneminyaka engu-50. Kulokhu, izindlala ze-parathyroid zisebenza ngokweqile.

• Izimpawu ezincane: ubuhlungu bamalunga, ubuthakathaka bemisipha, ukukhathala, ukucindezeleka, ubunzima bokugxilisa ingqondo, ukulahlekelwa yisifiso sokudla.
• Izimpawu ezibucayi: isicanucanu, ukuhlanza, ukudideka, ukukhohlwa, ukoma ngokweqile kanye nokuchama njalo, ukuqunjelwa, ubuhlungu emathanjeni.

• Ngenxa yezimila ku-pancreas kanye nengxenye yokuqala yamathumbu amancane (i-Gastrinomas):

Cishe ama-40% abantu abadala abane-MEN type 1 bahlakulela izimila ezibizwa ngokuthi ama-gastrinoma. Lawa akhiqiza i-hormone ebizwa ngokuthi i-gastrin, ebangela ukuthi isisu sikhiqize i-asidi eningi kakhulu.

• Izimpawu: Ubuhlungu besisu, isifo sohudo, isilungulela (i-acid reflux), izilonda zesisu (izilonda ze-peptic).
• Esinye isimila esibonakala kulesi sigaba yi -insulinoma . Lokhu kukhiqiza i-insulin, engabangela ushukela wegazi ophansi (hypoglycemia).
• Izimpawu: ukudideka, ukuthuthumela, ukujuluka, indlala enkulu, ukungaphumuli, ukushaya kwenhliziyo okusheshayo, izinguquko zombono zesikhashana.

• Ngenxa yezimila ze-pituitary (ama-prolactinoma):

Cishe abantu abangu-25% abane-MEN type 1 bahlakulela izimila ze-pituitary. Ezivame kakhulu yi -prolactinomas, ezikhiqiza i-hormone i-prolactin .

• Izimpawu kwabesifazane: ukungahlali kahle esikhathini noma ukuyeka ukuya esikhathini (i-amenorrhea), ubunzima bokukhulelwa izingane, ukuphuma kobisi ezingonweni lapho ungakhulelwe noma ungancelisi (i-galactorrhea), ukwehla kwesifiso sobulili.
• Izimpawu kwabesilisa: Ukwehla kwesifiso sobulili ngenxa yokwehla kwamazinga e-testosterone, ukungasebenzi kahle kwe-erectile (ED), kanye nobunzima bokukhulelwa izingane.
• Uma iqhubu likhulu: ikhanda elibuhlungu, isicanucanu/ukuhlanza, ukushintsha kombono (ukubona kabili, ukwehla kokubona eceleni).

Okubalulekile: Akuwona wonke umuntu ozoba nezimpawu ezifanayo. Lolu hlu aluphelele. Kunezinhlobo eziningi zezigaxa ezingavela ku-`(MEN type 1)`.

Ake sifunde futhi ngohlobo `(MEN)` (`MEN uhlobo 2`)

Wonke umuntu onesifo sohlobo lwe-MEN 2 uba nohlobo lomdlavuza we-thyroid olubizwa ngokuthi umdlavuza we-thyroid we-medullary (MTC) . Lolu hlobo lomdlavuza luba nohlobo olukhethekile lweseli endlaleni ye-thyroid ebizwa ngokuthi amaseli e-C. Lawa maseli akhiqiza i-hormone ebizwa ngokuthi i-calcitonin. I-MTC ingasakazekela kuma-lymph node nakwezinye izitho zomzimba. Ukwelashwa okuyinhloko ukususwa kwendlala ye-thyroid ngokuhlinzwa (i-thyroidectomy).

Futhi, abantu abane-`(MEN type 2)` bangase babe nesinye noma zombili zezimo ezilandelayo:

• I-Pheochromocytoma: Lesi yisisu esingavamile esikhula engxenyeni ephakathi yesinye noma zombili izindlala ze-adrenal (i-adrenal medulla). Esikhathini esiningi, lezi azinawo umdlavuza. Cishe u-10%-15% wamacala angaba umdlavuza futhi asakazeke.
• I-Hyperparathyroidism: Amazinga e-calcium egazini ayanda ngenxa yokukhiqizwa ngokweqile kwe-hormone ye-parathyroid (PTH) yizindlala ze-parathyroid.

Ziyini izimpawu ze-`(MEN type 2)`?

Izimpawu lapha nazo zihlukile. Izimpawu zivame ukubangelwa amazinga aphezulu ama-hormone akhiqizwa umdlavuza (i-MTC) kanye nezinye izimila.

• Izimpawu zomdlavuza we-thyroid we-medullary (MTC):
• Iqhubu elingaphambili entanyeni, ubuhlungu entanyeni, ukushintsha kwezwi (ukuhosha), ukukhwehlela, ubunzima bokugwinya, ubunzima bokuphefumula.

• Izimpawu ezibangelwa yi-Pheochromocytoma: (zenzeka cishe kuma-50% wamacala)
• Izimpawu zenzeka kuphela uma lezi zimila zikhiqiza ama-hormone amaningi kakhulu, njenge-adrenaline. Amanye ama-tumors awakhiqizi ama-hormone futhi angase angakhombisi zimpawu.
• Izimpawu ezivamile (ngokuvamile zifika ziphinde zidlule ngeziphonso): umfutho wegazi ophakeme (umfutho wegazi ophakeme), ikhanda elibuhlungu, ukujuluka ngokweqile ngaphandle kwesizathu, ukushaya kwenhliziyo okusheshayo noma okungajwayelekile, ukuthuthumela.

• Izimpawu ezibangelwa yi-hyperparathyroidism:
• Njengaku-`(MEN type 1)`, izimpawu ezifana nobuhlungu bamalunga, ubuthakathaka bemisipha, ukukhathala, ukucindezeleka, ubunzima bokugxilisa ingqondo, ukulahlekelwa yisifiso sokudla, isicanucanu, ukuhlanza, ukoma ngokweqile kanye nokuchama njalo, ukuqunjelwa, kanye nobuhlungu bamathambo kungenzeka.

Ubani ongaba nalesi simo '(AMADODA)'? Sivame kangakanani?

Lesi simo `(MEN)` singathinta abesilisa nabesifazane ngokulinganayo. Ubudala lapho lesi simo `(MEN uhlobo 1)` sivela khona buyahlukahluka kakhulu. Sitholakale ezinganeni ezineminyaka engu-8 ubudala kanye nabantu abadala abaneminyaka engu-80 ubudala.

`(AMADODA)` yisimo esingavamile .

• `(Uhlobo 1 lwamadoda)` luthinta cishe umuntu oyedwa kwabangu-30,000.
• `(MEN type 2)` ithinta cishe umuntu oyedwa kwabangu-35,000.

Abanye abacwaningi bakholelwa ukuthi inani langempela labantu abanalezi zimo lingase libe phezulu ngenxa yokungahlolwa kahle noma ukuhlonza okungalungile.

Odokotela basithola kanjani isimo `(MEN)`? (Ukuxilongwa)

Ukuxilonga isimo `(MEN)` kungaba nzima kancane, ngoba kuthinta izindlala ezahlukene.

• Ukuhlonza `(Uhlobo 1 LWE-MEN)`:

Umuntu uvame ukutholakala ene-MEN type 1 uma okungenani enezimila ezimbili kwezithathu ezinkulu ze-endocrine ezishiwo ngenhla (izimila ze-parathyroid, izimila ze-pituitary, kanye/noma izimila zomgudu wokugaya ukudla), noma uma enesinye salezo zimila futhi ilungu lomndeni line-MEN type 1.

• Ukuhlolwa:
• Ukuhlolwa kwegazi: Hlola amazinga aphezulu ama-hormone athile (isib. i-parathyroid hormone (PTH), i-calcium).
• Ukuhlolwa kokuthatha izithombe: Lokhu kungasiza ekutholeni izindawo ezinamaqhubu, njenge-CT scan (i-computed tomography scan) noma i-MRI (i-magnetic resonance imaging scan).
• Ukuhlolwa kwezakhi zofuzo: Kuhlolwa ukuguquka kwezakhi zofuzo ze-`MEN1`.

• Ukuhlonza `(Uhlobo 2 LWE-MEN)`:

Umuntu utholakala ene-MEN type 2 uma enomdlavuza we-thyroid we-medullary (MTC) kanye ne-pheochromocytoma kanye/noma i-hyperplasia noma i-adenoma yezindlala ze-parathyroid.

• Ukuhlolwa:
• Ukuhlolwa kwegazi: Hlola amazinga ama-hormone njenge-calcitonin (ye-MTC), i-parathyroid hormone (PTH), kanye ne-catecholamines (ye-pheochromocytoma).
• Ukuhlolwa kwezithombe: `i-CT scan` noma `i-MRI scan` kuyasetshenziswa.
• Ukuhlolwa kwezakhi zofuzo: Kuhlolwa ukuguquka kwezakhi zofuzo ze-'RET'.

Yiziphi izindlela zokwelapha `(AMADODA)`?

Ukwelashwa kwe-MEN kuncike ngokuphelele ekutheni yiziphi izindlala ze-endocrine nezitho zomzimba ezithintekile. Ngokuvamile kuhilela ithimba lodokotela elihlanganisa imikhakha eyahlukene, okuhlanganisa odokotela be-endocrinologists , odokotela abahlinzayo , kanye nodokotela be-oncologists .

Kungase kube nezinketho eziningana zokwelapha:

• Imithi: Lawula izimpawu futhi unciphise imiphumela yama-hormone amaningi.
• Ukuhlinzwa: Susa iqhubu noma yonke indlala ethintekile (isb., indlala yegilo).
• Ukwelashwa kokushintsha ama-hormone: Uma i-endocrine gland isuswa ngokuhlinzwa, ama-hormone akhiqizwa yiyo anikezwa ngaphandle.
• Ukwelashwa komdlavuza: Uma umdlavuza ususakazekele kwezinye izindawo (osuhlukaniswe ngama-metastases), ukwelashwa okufana nokwelashwa ngamakhemikhali kanye nokwelashwa ngemisebe .

Ngenxa yokuthi isiguli ngasinye `(SAMAN)` sihlukile, uhlelo lokwelapha olusebenzela wonke umuntu luhlukile. Ungesabi ukukhuluma nodokotela wakho ngezinketho zokwelashwa ezikulungele.

Ingabe `(AMADODA)` angelapheka ngokuphelele? Ingabe angavinjelwa?

Ngeshwa, okwamanje alikho ikhambi lalesi simo `(MEN)`. Kodwa-ke, singaphathwa . Odokotela baphatha izinguquko endlaleni ngayinye ngokuhlinzwa noma ngemithi efanele ngaleso sikhathi.

Akunakwenzeka ukuvimbela ukuthuthukiswa kwe-`(MEN)`, ngoba kubangelwa ukuguquka kwezakhi zofuzo. Lokhu kuguquka kwezakhi zofuzo kungazuzwa kubazali noma kwenzeke ngokungahleliwe ngesikhathi sesigaba sombungu.

Kodwa-ke, uma ilungu lomndeni eliseduze (umzali, umfowabo noma udadewabo) litholakale linesifo i-MEN, kubalulekile ukukhuluma nodokotela wakho mayelana nokuhlolwa kofuzo kwe-MEN. Ukwenza kanjalo kungasiza ekutholeni izimila kusenesikhathi uma unalesi simo.

Kwenzekani uma uhlala `(nabesilisa)`? (Isibikezelo)

Ukubikezela kwe-MEN kuncike ezintweni eziningana:

• Indlela okusheshe ngayo ukutholakala ukuthi `(MEN)`.
• Yiziphi izindlala ze-endocrine ezithintekayo?
• Kungakhathaliseki ukuthi iqhubu linomdlavuza noma cha.
• Uhlobo lokwelashwa olusetshenzisiwe.
• Kubizwa ngokuthi isimila somdlavuza sisakazekele kwezinye izingxenye zomzimba (esihlukaniswe ngama-metastasized).

Uma utholakale une-MEN, udokotela wakho angakunikeza umbono omuhle kakhulu mayelana nokwelashwa kwakho kanye nendlela yokuqhubeka.

Isikhathi sokubona udokotela kanye nemibuzo ebalulekile okufanele uyibuze

Uma utholakale une-MEN, kuzodingeka ubone udokotela wakho njalo ukuze aqaphe isimo sakho futhi aqinisekise ukuthi ukwelashwa kwakho kusebenza kahle.

Futhi, uma ilungu lomndeni eliseduze emndenini wakho line-`(MEN)`, khuluma nodokotela wakho mayelana nokuhlolwa kofuzo, njengoba kushiwo ngaphambili.

Uma une-`(MEN)`, kungaba usizo ukubuza udokotela wakho imibuzo efana nale:

• Hlobo luni lwe-`(MEN)` enginalo?
• (MEN) iwuthinta kanjani umzimba wami?
• Yiziphi izimpawu okufanele ngiziqaphele?
• Yiziphi izindlela zokwelapha enginazo?
• Yiziphi izinzuzo kanye nokungalungi kwezindlela ezahlukene zokwelapha?
• Kuthatha isikhathi esingakanani ukuthi ukwelashwa kuphumelele?
• Ingabe wonke umndeni wami kufanele uhlolwe (AMADODA)?

Okokugcina, izinto okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

I-multiple endocrine neoplasia (MEN) iyisimo esingavamile. Futhi, ngenxa yokuthi AMADODA angabangela izimpawu eziningi ezahlukene futhi isimo sabo bonke asifani, ngezinye izikhathi kungaba nzima ukwazi ukuthi unaso yini.

Uma uthola noma yiziphi izimpawu ezintsha ezikukhathazayo, noma uma ubona noma yiziphi izinguquko emzimbeni wakho, into engcono kakhulu ongayenza ukubona udokotela.

Nakuba ezinye izimo ze-'(MEN)' zenzeka ngokungahleliwe, zingase zivele nasemindenini. Uma omunye wezihlobo zakho eziseduze ene-'(MEN)', kubaluleke kakhulu ukuthola ukuhlolwa kofuzo ukuze uthole ukuthi usengozini yini. Khuluma nodokotela wakho nganoma yimiphi imibuzo onayo mayelana nengozi yakho yokuthola i-'(MEN)'. Bakhona ukukusiza. Ungesabi, ungesabi, futhi ungesabi ukubuza imibuzo.

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