Chii chinonzi MEN-1? Zviratidzo, Zvinokonzera, & Kurapa

Multiple Endocrine Neoplasia Type 1 (MEN-1) chirwere chisiyana chemagene chinoita kuti zvimwe zvibereko zvikure muzvikamu zvakawanda zvinoburitsa mahomoni mumuviri wako. Aya maberekero kazhinji anenge asina kenza, asi anogona kukanganisa maitiro emuviri wako pakugadzira nekushandisa mahomoni.

Fungidzira sisitimu yako ye endocrine sechikamu chekutaurirana kwemuviri wako, ine zvikamu zvinoburitsa mahomoni kudzora zvinhu zvakawanda kubva kune shuga yeropa kusvika kune simba remapfupa. Kana uine MEN-1, iyi network inogona kukura zvibereko zvinokanganisa kugadzirwa kwehomoni, zvichiita kuti pave nezviratidzo zvakasiyana-siyana izvo zvingaita sekusina hukama pakutanga.

Chii chinonzi MEN-1?

MEN-1 chirwere chinogona kuwanikwa mumhuri chinotapurwa zvikuru muzvikamu zvitatu zvikuru zvemumuviri wako: parathyroid glands muhuro rako, pancreas, uye pituitary gland mubongo rako. Chirwere ichi chinonzi MEN-1 nekuti chinosanganisira zvikamu zvakawanda zve endocrine zvinokura neoplasia, izvo zvinoreva kukura kwetsinga isina kujairika.

Chirwere ichi chinotapurwa nevanhu vanenge 1 pavagumi ve30,000, zvichiita kuti chive chisiyana. Chinhu chinoita kuti MEN-1 ive yakaoma ndechekuti inogona kukonzera zviratidzo zvakasiyana-siyana muvanhu vakasiyana, kunyange mumhuri imwe chete.

Maberekero anobatanidzwa neMEN-1 anokura zvishoma nezvishoma. Kunyange zvazvo mazhinji acho asina kenza, anogona kukonzera matambudziko ehutano nekugadzira mahomoni akawanda kana kukura zvakakwana kudzvanya pamasangano ari pedyo.

Ndezvipi zviratidzo zveMEN-1?

Zviratidzo zveMEN-1 zvinogona kusiyana-siyana nekuti zvinoenderana nezvikamu zvakakanganiswa uye huwandu hwehomoni zvavari kuburitsa. Vanhu vakawanda havazivi kuti vane MEN-1 kusvika vava vakuru, sezvo zviratidzo zvichiwanzoitika zvishoma nezvishoma kwemakore.

Sezvo MEN-1 ichikanganisa parathyroid glands yako kazhinji, unogona kutanga kuona zviratidzo zvine chekuita ne calcium yakawanda mweropa rako:

• Matombo emuviri anogara achizouya zvakare
• Kurwadziwa kwepfupa kana kuputsika kunowanzoitika nyore nyore
• Kuneta kunorasikirwa nekuzorora
• Kusuruvara kana kuchinja kwemanzwiro
• Kushaya simba retsinga
• Nyota yakawanda uye kuenda kunzvimbo kazhinji
• Kurutsa kana kurwadziwa kwemudumbu
• Matambudziko ekurangarira kana kusanzwisisa

Kana MEN-1 ikakanganisa pancreas yako, unogona kuona zviratidzo zvine chekuita nekusawirirana kwehomoni ipapo. Kana maberekero anogadzira insulin akakura, unogona kuva nezviitiko zve shuga yakaderera mweropa ine zviratidzo senge kupererwa neperspiration, kudedera, kurova kwemoyo nekukurumidza, kana kunyange kupera simba.

Vamwe vanhu vanokura maberekero anogadzira gastrin, homoni inowedzera asidhi yemudumbu. Izvi zvinogona kukonzera maronda emudumbu akakomba, kupisa kwemoyo kunogara kwenguva refu, manyoka, kana kurwadziwa kwemudumbu kunopindura zvishoma pakurapa kwakajairika.

Kana pituitary gland ikabatwa, unogona kuona zviratidzo senge kurwadziwa kwemusoro kunogara kwenguva refu, kuchinja kwekuona, kana matambudziko ekukura uye kukura kwepabonde. Vamwe vanhu vanowana nguva dzisingawanzoitiki, kudzikira kwechido chepabonde, kana kuburitsa mukaka kusatarisirwa kubva kumabwerebwere.

Mumazuva mashoma, MEN-1 inogona kukanganisa zvimwe zvikamu zvemumuviri wako, zvichikonzera kukura kwe ganda, maberekero emumapfupa, kana maberekero mu adrenal glands. Aya matambudziko haawanzoitiki asi anogona kuitika sezvo chirwere ichi chichienderera mberi.

Chii chinokonzera MEN-1?

MEN-1 inokonzerwa nekusashanda zvakanaka kwe gene reMEN1, rinowanzo kubatsira kudzivirira maberekero kubva pakukura muzvikamu zvako zve endocrine. Kana iri gene isingashande zvakanaka, masero aya muzvikamu izvi anogona kukura zvisina kudzora, achigadzira maberekero anoratidza chirwere ichi.

Kuchinja uku kwemagene kunogona kuwanikwa mumhuri zvinotaurwa nachiremba kuti autosomal dominant pattern. Izvi zvinoreva kuti unongoda kuwana kopi imwe chete ye gene yakakanganiswa kubva kune mumwe wevabereki kuti ubude MEN-1. Kana mumwe wevabereki vako aine MEN-1, une mukana we50% wekuwana chirwere ichi.

Muhuwandu hwe10% yezviitiko, MEN-1 inoitika se mutation itsva, zvinoreva kuti hapana mumwe wevabereki ane chirwere ichi. Aya maitiro anonzi de novo mutations, uye anoitika zvisingatarisirwi panguva yekugadzira masero ekuberekesa kana kukura kwekutanga.

Gene reMEN1 rinogadzira protein inonzi menin, inoshanda senge murindi mumasero ako. Menin inobatsira kudzora kupararira kwemasero uye kudzivirira masero kubva pakukura nekukurumidza. Kana gene ikakuvara, iyi nzira yekudzivirira inokundikana, ichibvumira kukura kwe maberekero munyama inogadzira mahomoni.

Rini unofanira kuona chiremba nekuda kweMEN-1?

Unofanira kubata chiremba wako kana ukaona matombo emuviri anogara achizouya, kunyanya kana uri mudiki kana akaenderera mberi kunyangwe kurapwa. Matombo emuviri anowanzoitika pamwe chete nematambudziko epfupa kana kuneta kunogara kwenguva refu zvinogona kuratidza dambudziko ne parathyroid glands dzako.

Tsvaga rubatsiro rwekurapa kana uine zviitiko zvekupererwa neperspiration, kudedera, kurova kwemoyo nekukurumidza, kana kusanzwisisa izvo zvinogona kuratidza kudonha kwakakomba kweshuga yeropa. Aya maratidzo, kunyanya kana akaitika usati wadya, anoda kuongororwa nekukurumidza.

Rongedza musangano kana ukaona maronda emudumbu asingarapiki nekurapa kwakajairika, kana uine kupisa kwemoyo kunogara kwenguva refu pamwe chete nemanyoka uye kurwadziwa kwemudumbu. Iyi musanganiswa wezviratidzo inogona kuratidza maberekero anogadzira mahomoni mu pancreas yako.

Bata chiremba wako kana uine kurwadziwa kwemusoro kunogara kwenguva refu pamwe chete nekuchinja kwekuona, sezvo izvi zvingave zviratidzo zve pituitary tumor. Zvakafanana, kana ukaona kuchinja kusatarisirwa mumazuva ako, chido chepabonde, kana kuburitsa mukaka kubva kumabwerebwere, izvi zvinogona kuva zviratidzo zve pituitary involvement.

Kana uine nhoroondo yemhuri yeMEN-1 kana zvirwere zvine hukama, zvakakosha kutaura nezvekuongororwa kwemagene uye kuongororwa nechikurapa chako, kunyangwe usati uine zviratidzo. Kuonekwa pakutanga kunogona kubatsira kudzivirira matambudziko uye kutungamira kuongororwa kwakakodzera.

Ndezvipi zvinokonzera ngozi zveMEN-1?

Chinhu chikuru chinokonzera ngozi yeMEN-1 ndechekuva nemubereki ane chirwere ichi. Sezvo MEN-1 ichitevera autosomal dominant inheritance pattern, vana vevabereki vakabatwa vane mukana we50% wekuwana mutation yemagene.

Kuva nenhoroondo yemhuri yemaberekero asingawanzoitiki ane chekuita nemahomoni, kunyangwe asina kuongororwa zviri pamutemo seMEN-1, kunogona kuwedzera ngozi yako. Dzimwe nguva chirwere ichi hachizivikanwi mumhuri kwemazuva mazhinji, kunyanya kana zviratidzo zvichinyoro kana kuiswa kune zvimwe zvinokonzera.

Kusiyana nezvimwe zvirwere zvakawanda, MEN-1 haina zvinokonzera ngozi zvine chekuita nemararamiro. Kudya kwako, tsika dzekurovedza muviri, zvinhu zvakakomberedza, kana kusarudza kwako hakusi kukanganisa kana uchakura chirwere ichi chemagene.

Zera rinogona kukanganisa nguva iyo zviratidzo zvinoonekwa, asi harichinji ngozi yako yekuva nechirwere ichi. Vanhu vakawanda vane MEN-1 vanokura zviratidzo pakati pemakore makumi maviri nemakumi mana, kunyange vamwe vangasaratidza zviratidzo kusvika gare gare muhupenyu kana, zvisingawanzoitiki, panguva yehudiki.

Rudzi harukanganisi zvakanyanya ngozi yako yekuwana MEN-1, kunyange zvimwe zviratidzo zvingaratidzika zvakasiyana muvarume nevakadzi nekuda kwekusiyana kwehomoni.

Ndezvipi zvinogona kuitika matambudziko eMEN-1?

Matambudziko eMEN-1 anogona kuva akakomba uye kazhinji ane chekuita nemigumisiro yehupenyu hurefu hwekusawirirana kwehomoni kana kuvapo kwemuviri kwemaberekero anokura. Kunzwisisa aya matambudziko anogona kuitika kunobatsira iwe nechikwata chako chekurapa kuramba muri vakangwarira nezvematambudziko.

Calcium yakawanda kubva ku parathyroid glands dzinoshanda zvakanyanya inogona kukonzera matambudziko akawanda anotyisa:

• Osteoporosis yakakomba uye kuwedzera kengozi yekuputsika
• Chirwere cheitsvo chekare kubva kumatombo emuviri anogara achizouya
• Matambudziko emwoyo kubva kune kusawirirana kwe calcium
• Kusuruvara kwakakomba kana kusakwanisa kufunga zvakanaka
• Maronda emudumbu anogona kupwanya kana kubuda ropa

Maberekero ePancreatic anogona kukonzera kudonha kwakakomba kweshuga yeropa izvo zvinogona kukonzera kuita seizures, kuenda mukoma, kana kukuvara kwenguva refu kwemubongo kana zvisina kurapwa nekukurumidza. Mamwe maberekero ePancreatic anogadzira gastrin yakawanda, zvichikonzera maronda akakomba anogona kupwanya mudumbu kana ura.

Maberekero ePituitary anogona kukura zvakakwana kudzvanya pamasangano ari pedyo, zvichikonzera kurasikirwa kwekuona kwenguva refu kana kushomeka kwakakomba kwehomoni. Maberekero makuru ePituitary anogona zvakare kukonzera kuwedzera kumanikidzwa mukati memusoro wako, zvichikonzera kurwadziwa kwemusoro kunogara kwenguva refu nematambudziko eneurological.

Kunyangwe mazhinji emabereko eMEN-1 asina kenza, kune njodzi diki yekuti mamwe anogona kuva kenza nekufamba kwenguva. Maberekero ePancreatic ane njodzi yakakwira yekushanduka kwakaipa, ndosaka kuongororwa nguva dzose kuri kukosha kwazvo.

Mumazuva mashoma, MEN-1 inogona kukonzera maberekero emumapfupa, thymus, kana adrenal glands. Aya matambudziko asingawanzoitiki anogona kuva akakomba uye anogona kuda nzira dzekurapa dzakasarudzika.

MEN-1 inozwivikanwa sei?

Kuziva MEN-1 kunosanganisira musanganiswa wekuongororwa kwezviratidzo, bvunzo dzeropa, kuongororwa kwemafirimu, uye kuongororwa kwemagene. Chiremba wako achatanga nekutarisa zviratidzo zvako nenhoroondo yemhuri kuti aone kana MEN-1 iri mukana.

Bvunzo dzeropa dzakakosha pakuzivisa uye kazhinji dzinosanganisira kuongorora calcium, parathyroid hormone, uye mahomoni akasiyana-siyana epancreatic. Calcium yakawanda ine parathyroid hormone yakawanda kazhinji inopa chiratidzo chekutanga chekuti MEN-1 ingave iripo.

Chiremba wako anogona kuraira bvunzo dzemahomoni zvakawanda, kusanganisira gastrin, insulin, glucagon, uye mahomoni e pituitary. Aya maitiro anobatsira kuziva kuti ndeapi zvikamu zvakakanganiswa uye zvakanyanya sei zvavari kuburitsa mahomoni.

Kuongororwa kwemafirimu kunobatsira kuwana maberekero uye kuona saizi yavo. Unogona kuva ne CT scans, MRI scans, kana kuongororwa kwakasarudzika kwe nuclear medicine kunogona kuona maberekero madiki anogadzira mahomoni ayo angasaratidzike pane kuongororwa kwakajairika.

Kuongororwa kwemagene kunogona kusimbisa kuziva nekutsvaga mutations mu gene reMEN1. Iyi bvunzo yakakosha zvikuru kuvanhu vemhuri vanoda kuziva kana vakaunza mutation yemagene, kunyangwe vasina zviratidzo.

Kuziva kunowanzosimbiswa kana uine maberekero mune zvikamu zvitatu zvikuru zvinangwa (parathyroid, pancreas, kana pituitary) kana kuongororwa kwemagene kuchiratidza mutation ye gene reMEN1.

Kurapa kweMEN-1 ndekupi?

Kurapa kweMEN-1 kunotarisa pakudzora kusawirirana kwehomoni uye kugadzirisa maberekero mumwe nemumwe sezvaanoonekwa. Sezvo iri chirwere chehupenyu hwese, chirongwa chako chekurapa chichava chinowanzochinja nekufamba kwenguva sezvo maberekero matsva achionekwa kana aripo achikura.

Kune parathyroid glands dzinoshanda zvakanyanya, kuvhiya kazhinji ndiyo kurapa kwakasarudzwa. Chiremba wako wekuvhiya anowanzo bvisa matatu nehafu eparathyroid glands dzako ina, achisiya tishu yakakwana kuchengetedza mazinga e calcium akajairika nepo achivhara chirwere kubva pakudzoka nekukurumidza.

Maberekero ePancreatic anoda kurapwa kwakasarudzika zvichienderana nehukuru hwavo, nzvimbo, uye kugadzira mahomoni. Maberekero madiki, asingashandi, anogona kungoongororwa, nepo maberekero makuru kana anogadzira mahomoni kazhinji anoda kubviswa nekuvhiya.

Kana uine maberekero anogadzira insulin achiita kuti shuga yeropa idonha zvakanyanya, chiremba wako anogona kupa mishonga senge diazoxide kubatsira kuchengetedza mazinga eshuga yeropa akatsiga. Kune maberekero anogadzira gastrin, proton pump inhibitors inogona kubatsira kudzora kugadzirwa kwe asidhi yemudumbu.

Maberekero ePituitary kazhinji anorapwa nemishonga inogona kudzikisa mamwe marudzi emabereko kana kudzivirira kugadzirwa kwehomoni yavo. Kuvhiya kunogona kudiwa kune maberekero makuru kana asingapinduri kumishonga.

Chikwata chako chekurapa chichava chinosanganisira nyanzvi dzakawanda, kusanganisira endocrinologists, ma surgeon, uye zvichida oncologists. Kuongororwa nguva dzose nebvunzo dzeropa uye mafirimu kunobatsira kubata matambudziko matsva pakutanga kana zviri nyore kurapa.

Maitiro ekugadzirisa MEN-1 pamba?

Kurarama neMEN-1 kunoda kutarisirwa kwenguva refu kune hutano hwako, asi kune zvinhu zvakawanda zvaunogona kuita pamba kubatsira hutano hwako uye kushanda zvakanaka nechikwata chako chekurapa.

Chengetedza rekodhi yakadzama yezviratidzo zvako, kusanganisira nguva yavanouya, kukomba kwavo, uye chero zvinogona kuvakonzera. Iyi ruzivo inobatsira vanachiremba vako kugadzirisa chirongwa chako chekurapa uye kubata matambudziko matsva pakutanga.

Kana uine matambudziko ane chekuita ne calcium, gara wakanyorova nekudhakwa kwemvura yakawanda zuva rese. Izvi zvinobatsira kudzivirira matombo emuviri uye kubatsira basa reitsvo rako. Dzivisa calcium supplements yakawanda kunze kwekunge yakurudzirwa zvakanyanya nachiremba wako.

Kudzora shuga yeropa, dyisa zvokudya zvakajairwa, zvakaringana, uye ubate glucose tablets kana zvokudya kana uchida kudonha kweshuga yeropa. Dzidza kuziva zviratidzo zvekutanga zve hypoglycemia kuitira kuti ugone kuzvirapwa nekukurumidza.

Chengetedza hutano hwepfupa hwakanaka nekurovedza muviri nguva dzose, uye uve nechokwadi chekuti une vitamin D yakakwana. Nekudaro, taura nezvechero zvinowedzerwa nechikwata chako chekurapa, sezvo zvinodiwa zve calcium zvinogona kusiyana nevanhu vakawanda.

Gadzira kadhi rekuziva hutano rinotaura nezvechirwere chako, mishonga iripo, uye vanhu vanogona kubatwa kana uine dambudziko. Chengetedza izvi newe nguva dzose, sezvo zvingave ruzivo rwakakosha kune vanachiremba kana uchida rubatsiro rwekukurumidza.

Unofanira kugadzirira sei musangano wako nachiremba?

Kugadzirira zvakakwana kune misangano yako kunobatsira kuve nechokwadi chekuti unowana zvakanyanya kubva panguva yako nechikwata chako chekurapa uye usakanganwa mibvunzo yakakosha kana zvinotyisa.

Nyora zviratidzo zvako zvose, kusanganisira nguva zvakatanga, kazhinji zvinoitika, uye zvinodzibatsira kana kuzviita zvakanyanya. Sanganisira zviratidzo zvingaita sekusina hukama, sezvo MEN-1 inogona kukonzera matambudziko akasiyana-siyana mumuviri wako.

Uya nerondedzero yakazara yemishonga yose, zvinowedzerwa, uye mavhitamini auri kutora, kusanganisira zvikamu uye kazhinji unotora. Izvi zvinosanganisira mishonga inowanikwa pasina kuraira, sezvo imwe inogona kukanganisa kurapwa kweMEN-1.

Unganidza nhoroondo yemhuri yako yezvehutano, kunyanya kuisa pfungwa pane hama dzakanga dzine matambudziko emahomoni, maberekero asingawanzoitiki, matombo emuviri, kana maronda emudumbu. Iyi ruzivo inogona kuva yakakosha pakuzivisa uye kuronga kurapa.

Gadzira rondedzero yemibvunzo nezvechirwere chako, sarudzo dzekurapa, uye zvaunofanira kutarisira kuenda mberi. Usazvidya mwoyo nezvekubvunza mibvunzo yakawanda - chikwata chako chekurapa chinoda kuti unzwisise chirwere chako zvizere.

Funga kuuya nemumwe wemhuri kana shamwari yaunovimba nayo kumusangano wako. Vanogona kukubatsira kuyeuka ruzivo rwakakosha uye kupa rutsigiro rwemanzwiro panguva yehurukuro inogona kunge yakanyanya nezvehutano hwako.

Chii chinonyanya kukosha nezveMEN-1?

MEN-1 chirwere chakaoma asi chinogona kudzorwa chemagene chinotapurwa muzvikamu zvinoburitsa mahomoni mumuviri wako. Kunyange zvazvo ichida kuongororwa kwenguva refu nekurapa, vanhu vakawanda vane MEN-1 vanorarama hupenyu hwazvo, hwakazara, nekuchengetedzwa kwakakodzera kwehutano.

Kuziva pakutanga uye kuongororwa kwenguva dzose kwehutano zvakakosha pakudzivirira matambudziko akakomba. Chinhu chikuru ndechekushanda pedyo nechikwata chako chekurapa kuti uongorore maberekero matsva uye utonge aripo zvinobudirira.

Kana uine MEN-1 kana uchifunga kuti ungave nayo, yeuka kuti chirwere ichi chinotapurwa nemunhu wese zvakasiyana. Chiitiko chako chingave chisingafaniri nechaunoverenga nezvevamwe, uye izvozvo zvakajairika zvachose.

Kuva neMEN-1 zvinoreva kuti uchada kutarisirwa kwakawanda kwehutano kupfuura vanhu vakawanda, asi hazvitsananguri hupenyu hwako. Nekurapwa kwakakodzera uye kuongororwa, unogona kudzora zviratidzo zvako uye kuchengetedza mhando yehupenyu hwako.

Mibvunzo inowanzo bvunzwa nezveMEN-1

Q.1: MEN-1 inogara ichinakirwa kubva kuvabereki here?

Anenge 90% yezviitiko zveMEN-1 zvinonakirwa kubva kune mubereki ane chirwere ichi. Nekudaro, anenge 10% yezviitiko zvinoitika semutations itsva, zvinoreva kuti hapana mubereki ane MEN-1. Kana uine MEN-1, mumwe nemumwe wevana vako ane mukana we50% wekuwana mutation yemagene.

Q.2: Maberekero eMEN-1 anogona kuva kenza here?

Mazhinji emabereko eMEN-1 anoramba asina kenza muhupenyu hwako hwese, asi kune njodzi diki yekushanduka kwakaipa, kunyanya nemaberekero epancreatic. Ndosaka kuongororwa nguva dzose nekutarisirwa kwenguva refu kuri kukosha kwazvo. Kuonekwa pakutanga kwechero kuchinja kunobvumira kurapwa nekukurumidza kana zvichidiwa.

Q.3: Kazhinji sei ndinofanira kuongororwa kana ndine MEN-1?

Vanhu vakawanda vane MEN-1 vanoda kuongororwa gore negore kana makore maviri ekuongororwa anowanzoita bvunzo dzeropa uye kuongororwa kwemafirimu. Huwandu chaihwo hunoenderana nezviratidzo zvako zviripo, zvikamu zvakakanganiswa, uye kuti chirwere chako chakatsiga sei. Chikwata chako chekurapa chichagadzira chirongwa chekuongororwa chakanyanya kukodzera iwe.

Q.4: Kuchinja kudya kana mararamiro kunogona kubatsira kudzora MEN-1 here?

Kunyangwe kuchinja mararamiro kuchingakwanisi kurapa MEN-1 kana kudzivirira kukura kwemaberekero, kunogona kubatsira kudzora zviratidzo uye kubatsira hutano hwako hwese. Kugara wakanyorova kunobatsira kudzivirira matombo emuviri, zvokudya zvakajairwa zvinobatsira kudzora shuga yeropa, uye kurovedza muviri kwakakodzera kunotsigira hutano hwepfupa. Nekudaro, kurapwa kwemishonga kunoramba kuri nzira huru.

Q.5: Hama dzangu dzinofanira kuongororwa zveMEN-1 here?

Kana uine MEN-1 yakasimbiswa, hama dzako dzepedyo (vabereki, vanun'una, nevana) dzinofanira kutaura nezvekuongororwa kwemagene nevanachiremba vavo. Kunyangwe vasina zviratidzo, kuziva mamiriro avo emagene kunobvumira kuongororwa kwakakodzera uye kuita chimwe chinhu pakutanga kana zvichidiwa. Kuongororwa kwemagene kunogona kubatsira mhuri kuita sarudzo dzakachenjera nezvekuongororwa.