Iyini i-Glucagonoma? Ake sixoxe ngalesi simo esingavamile!

I-pancreas iyisitho esincane kodwa esibaluleke kakhulu emzimbeni wakho. Ikhiqiza ama-hormone amaningana adingwa umzimba wethu. Ngakho-ke, ngezinye izikhathi, uhlobo olungavamile lomdlavuza oluvela ku-pancreas lungabangela izinkinga. Yilokho lesi simo esibizwa ngokuthi i-Glucagonoma . Mhlawumbe usuke waba nesifo sikashukela ngokuzumayo, noma usuke waba nokuqubuka okungajwayelekile esikhumbeni sakho, kubaluleke kakhulu ukuqaphela lokhu.

Iyini i-glucagonoma? Kalula nje...

I-Glucagonoma iyisimila esingavamile kakhulu esinomdlavuza esikhula ku-pancreas yakho. Lesi simila sikhiqiza i-hormone eningi kakhulu ebizwa ngokuthi i-glucagon. Manje ungase uzibuze ukuthi iyini le-glucagon.

I-Glucagon iyi-hormone ebaluleke kakhulu esiza ukulawula izinga likashukela emzimbeni wethu, okungukuthi, izinga likashukela egazini . Yenziwa amaseli e-alpha, uhlobo olukhethekile lweseli kuma-pancreas akho. Cabanga uma ungakadli isikhathi eside, uma uvivinya umzimba kanzima, noma uma udla ukudla okucebile ngamaprotheni, izinga likashukela egazini lakho lingase lehle, akunjalo? Yilapho le glucagon ingena khona, ikhipha ushukela ogcinwe esibindini sakho, okungukuthi, i-glycogen , futhi ibuyisele izinga likashukela egazini phezulu. Kufana nomuntu osiza ekugcineni ibhalansi kashukela emzimbeni wethu.

Kodwa-ke, uma kukhona i-glucagonoma, le hormone ye-glucagon ikhiqizwa ngokweqile. Khona-ke umzimba wethu awukwazi ukuyiphatha. Inhlanganisela yezimpawu ezenzeka ngenxa yalokhu ibizwa ngokuthi i-Glucagonoma Syndrome . Lokhu kungaholela ezinkingeni eziningi ezifana nezinkinga zesikhumba nesifo sikashukela.

Lolu hlobo lomdlavuza, olubizwa ngokuthi i-glucagonoma, lungolwesigaba sama-pancreatic neuroendocrine tumors , noma ama-islet cell tumors . Ngokuvamile, lawa avela emsileni noma engxenyeni ephakathi ye-pancreas.

Ingabe lo umdlavuza ngempela?

Yebo, isikhathi esiningi, i-glucagonoma ingumdlavuza . Into edabukisayo ukuthi, ngisho noma itholakala, cishe emacaleni angu-50%, okungukuthi, cishe oyedwa kwababili, lo mdlavuza kungenzeka ukuthi ususakazeke (wasakazeka) kwezinye izingxenye zomzimba, ikakhulukazi isibindi .

Sivame kangakanani lesi simo?

I-Glucagonoma empeleni iyisimo esingavamile kakhulu . Kutholakala abantu abasha abangaphansi kwesisodwa kwabayisigidi minyaka yonke. Ivame ukuthinta abantu abaneminyaka ephakathi kuka-50 no-70 .

Ziyini izimpawu zalokhu?

Ama-glucagonoma avame ukukhula kancane . Ngakho-ke, ngaphandle kokuthi umzimba wakho ukhiqize i-glucagon eyanele ukubangela izinkinga, ungase ungabi nazimpawu.

Kodwa-ke, uma i-glucagonoma ikhiqiza i-glucagon eningi kakhulu futhi umzimba ungakwazi ukuyiphatha, kuvela isifo se-glucagonoma okukhulunywe ngaso ngaphambili.Kuyenzeka. Ngemuva kwalokho, uzobona izimpawu ezicacile. Uma unalezi zimpawu, kubaluleke kakhulu ukubona udokotela ngokushesha okukhulu.

Ukuqubuka kwesikhumba (i-Necrolytic Migratory Erythema - NME)

Lolu uphawu oluyinhloko noluvame kakhulu lwe-glucagonoma syndrome. Cishe abantu abangu-90% abanalesi sifo bahlushwa yile nkinga yesikhumba. Ibizwa ngokuthi i-necrolytic migratory erythema (NME) .

Cabanga nje, isikhumba sakho, ikakhulukazi ezingxenyeni zangasese, ezinqeni, nasemilenzeni engezansi, siqala ukuba namabala abomvu, alumayo, futhi ngezinye izikhathi abuhlungu. Lawa kamuva aphenduka amabhamuza, aqhekeke, futhi abe uqweqwe. Ngisho nangemva kokuba epholile, ibala elinsundu lingasala. Lokhu kungakheka endaweni eyodwa, kuphole, bese kuvela futhi kwenye indawo. Kufana nokuqubuka okuhamba kusuka kwenye indawo kuya kwenye.

Ezinye izici ze-NME:

• Lokhu kungafika kudlule, futhi kungahambahamba emzimbeni .
• Kungaba lusizi kakhulu futhi kube buhlungu ekuqaleni.
• Ukuqhuma kuba bomvu bese kuba amabhamuza bese kuba uqweqwe. Ngemva kokuphola, kungase kuhlale ibala elinsundu.
• Lokhu kungaholela ezintweni ezifana nezilonda zolimi (i-glossitis ), izilonda zomlomo, izindebe eziqhekekile, kanye ne-angular cheilitis .
• Ukuvuvukala kwamehlo ( i-blepharitis ), ukulahleka kwezinwele, kanye nezinkinga zezinzipho nakho kungase kwenzeke.

Izimpawu zesifo sikashukela

Lesi sifo sibangela amazinga aphezulu kashukela egazini ( i-hyperglycemia ), okungaholela kushukela. Izimpawu zesifo sikashukela zifaka:

• Ukuba mncane ngaphandle kwesizathu.
• Ukoma ngokweqile njalo (i-Polydipsia) .
• Ukuchama njalo ( i-polyuria ), ikakhulukazi ebusuku.
• Indlala eningi ( i-Polyphagia ).

Ezinye izimpawu ze-(glucagonoma syndrome)

Kunezinye izimpawu eziningana:

• Uhudo olungapheli .
• I-hemoglobin ephansi egazini (i-anemia ). Lokhu kungabangela ukukhathala kanye nesiyezi.
• Amahlule egazi, ikakhulukazi emithanjeni ejulile yemilenze ( i-Deep Vein Thrombosis (DVT) ).

Izimpawu ezihlobene nohlelo lwengqondo nolwezinzwa

Ngezinye izikhathi, ungase ubone nezimpawu ezihlobene nohlelo lwengqondo nolwezinzwa, njenge:

• Ukucindezeleka
• Isifo sokulahlekelwa yingqondo
• Ukucasuka
• I-Hyperreflexia (ukusheshisa izimpendulo zomzimba)
• Ukuhamba okuxakile (ataxia )
• Izinkinga zengqondo ( Psychosis))
• Ukwesaba nokusola okungenangqondo ( I-Paranoid Delusions )

Yini ebangela i-glucagonoma?

Esikhathini esiningi, odokotela abazi imbangela eqondile ye-glucagonoma. Kodwa-ke, cishe ezimweni eziyi-10%, lo mdlavuza uhlotshaniswa nesimo sofuzo esibizwa ngokuthi i-Multiple Endocrine Neoplasia type 1 (MEN type 1) .

Kodwa-ke, akubona bonke abantu abane-MEN type 1 abathola i-glucagonoma. Nalokho kuyiphesenti elincane kakhulu.

Ukubona kanjani lokhu?

Uma une -necrolytic migratory erythema (NME) yesifo sesikhumba okukhulunywe ngayo ngaphambili, udokotela angase asole i-glucagonoma. Bangase benze izivivinyo ezifana nalezi:

• Ukuhlolwa Koshukela Ogazini Ozila Ukudla: Uma izinga likashukela liphezulu, i-glucagonoma ingasolwa futhi isifo sikashukela esihambisana nayo singaqinisekiswa.
• Ukuhlolwa Kwegazi le-Glucagon Yokuzila Ukudla: Amazinga e-Glucagon aphezulu ngendlela engavamile ku-glucagonoma - ngokuvamile angaphezu kwama-picogram angu-500 nge-milliliter (pg/mL). Amazinga ajwayelekile e-glucagon yokuzila ukudla angaphansi kuka-150 pg/mL.
• Ukuhlolwa Kwegazi Kwe-Amino Acid: I-Glucagonoma ivame ukubangela amazinga aphansi ama-amino acid egazini ( i-hypoaminoacidemia ).
• Ukubalwa Kwegazi Okuphelele (i-CBC): Lokhu kuhlola i-anemia , njengoba i-anemia ingenzeka nge-glucagonoma.

Ukuhlolwa kwezithombe ezinjengalezi kungasiza ekuqinisekiseni ukuthi kukhona yini iqhubu:

• I-CT Scan
• Iskeni se-MRI
• I-Ultrasound ye-Endoscopic

Iphathwa kanjani?

Ukwelashwa kwe-glucagonoma kuncike kakhulu ekutheni umdlavuza ukhawulelwe ku-pancreas (osendaweni) noma usakazekele kwezinye izingxenye zomzimba (osuvele usakazekile) .

Indlela yokulawula izimpawu kuqala

Isinyathelo sokuqala ukulawula izimpawu nezinkinga ezibangelwa ukweqisa kwe-glucagon. Lokhu kuphathwa kanje:

• Imithi yokulawula i-glucagon eningi: Imijovo ebizwa ngokuthi i-Octreotide kanye ne-Lanreotide isiza ukunciphisa imiphumela ye-glucagon eningi futhi imise ukukhiqizwa kwe-glucagon. Le mithi inganciphisa nokukhula kwezimila. Iphinde inikeze impumuzo ezimpawini ze-NME, isifo sikashukela, isifo sohudo, kanye nezinkinga zesimiso sezinzwa.
• Ukuphathwa kwesifo sikashukela: Ungadinga imithi yokwelapha isifo sikashukela noma imijovo ye-insulin ukuze ugcine amazinga kashukela egazini lakho esezingeni elifanele.
• Ukusekelwa kokudla okunempilo:I-Total Parenteral Nutrition (TPN) , ama-amino acid kanye nezithasiselo ze-zinc zinganikezwa ukunciphisa imiphumela yokungondleki kahle.
• Ukwelashwa kwesifo sesikhumba se-NME: Ama-antibiotic kanye nama-corticosteroids angathuthukisa i-NME.
• Ukwelashwa Ngemithi Yokulwa Nokuqhekeka Kwamagazi: Izinto zokunciphisa igazi ezifana ne-heparin zisiza ekuvimbeleni i-deep vein thrombosis (DVT) .

Uma kukhawulelwe kuma-pancreas...

Uma umdlavuza use-pancreas kuphela, ukuhlinzwa kuyindlela eyinhloko yokwelapha . Uma udokotela ohlinzayo engakwazi ukususa isimila ngokuphelele, lesi sifo sivame ukwelapheka . Ngemva kokususwa kwe-glucagonoma, amazinga akho e-glucagon kufanele abuyele esimweni esijwayelekile. Izimpawu zakho nazo kufanele ziphele.

Uma isisabalale kwezinye izindawo (i-metastasized)...

Noma nini lapho kungenzeka, odokotela batusa ukususwa kwezimila ngokuhlinzwa. Lokhu kungafaka phakathi ukususwa kwezimila ku-pancreas, ama-lymph node aseduze, kanye nesibindi.

Kodwa uma udokotela ohlinzayo engakwazi ukususa noma yiliphi iqhubu noma lonke, ithimba lakho lezokwelapha lingancoma eyodwa noma ngaphezulu yalezi zindlela zokwelapha:

• Ukwelashwa ngamakhemikhali: Lokhu kwelashwa kusebenza ngokubhubhisa amangqamuzana omdlavuza nokuwavimbela ekukhuleni kwawo.
• Ukususwa Kwemisebe: Lokhu kusebenzisa ukushisa okuvela emagagasini omsakazo ukubulala amangqamuzana omdlavuza.
• Ukususwa kwe-Cryosurgical: Lokhu kusebenzisa amakhemikhali abandayo kakhulu, njenge-nitrogen ewuketshezi, ukubhubhisa amangqamuzana omdlavuza.
• Ukwelashwa nge-Chemoembolization: Lokhu kwelashwa kusebenza ngokuvimbela ukutholakala kwegazi eliya esigaxeni somdlavuza.

Iyini iphrojekthi yokululama kulesi simo?

Umbono womuntu one-glucagonoma uncike ezintweni eziningana:

• Ubudala bakho kanye nempilo yakho iyonke .
• Usayizi wesimila somdlavuza .
• Kungakhathaliseki ukuthi umdlavuza ususakazeke noma cha .

Uma umdlavuza utholakala ku-pancreas kuphela, umbono uvame ukuba muhle . Ukuhlinzwa kokususa isimila kuvame ukwelapha, kodwa lokhu kwenzeka cishe kuma-20% eziguli ze-glucagonoma.

Ngenxa yokuthi i-glucagonoma ayivamile, ayikho idatha eyanele yokusho ukuthi ungaphila isikhathi esingakanani noma ukuthi umphumela uzoba yini uma umdlavuza usakazeka. Ithimba lakho lezokwelapha lizokwazi ukukunikeza ulwazi olwengeziwe ngokusekelwe esimweni sakho esithile.

Ingabe lokhu kungavinjelwa?

Ngenxa yokuthi ososayensi abazi imbangela yamacala amaningi e-glucagonoma, akukho lutho ongakwenza ukuze uvimbele lokhu .

Kodwa-ke, uma omunye wezihlobo zakho zezinga lokuqala (abazali bakho kanye nezingane zakini) etholakale ene -MEN (Multiple Endocrine Neoplasia) , khuluma nodokotela wakho mayelana nokuhlolwa kwezakhi zofuzo. Uma nawe une-MEN, ukuhlolwa kwezakhi zofuzo kungasiza ekutholeni umdlavuza ezigabeni zawo zokuqala.

Ukutholwa unomdlavuza kuyinto ecasulayo kakhulu. Uma kuyinto engavamile njenge-glucagonoma, kuba nzima nakakhulu. Kodwa khumbula, ithimba lakho lezokwelapha likhona ukuze liphendule imibuzo yakho futhi likusekele. Uma uzizwa uwedwa ngokwengqondo nangokomzwelo nge-glucagonoma, buza udokotela wakho ngezinhlelo nezinsizakalo ezingakusiza.

Okokugcina, izinto okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

Ngakho-ke, yize i-glucagonoma iyisimo esingavamile kakhulu, kubalulekile ukuqaphela. Ikakhulukazi uma ngokuzumayo uba nezimpawu ezifana nesifo sikashukela kanye nokuqubuka kwesikhumba okungajwayelekile (i-NME), njengoba kushiwo, bona udokotela ngokushesha. Uma kuqashelwa kusenesikhathi, kungelashwa. Nakuba lokhu kungavamile, kunezindlela zokwelapha.

Ungakhathazeki, awuwedwa. Udokotela wakho kanye nethimba lezokwelapha bazokusiza kulolu hambo. Hlala uqinile futhi ulandele iseluleko abakunikeza sona.

I- Glucagonoma, umdlavuza we-pancreatic, i-glucagon, isifo sikashukela, izimpawu zesikhumba, ama-hormone, umdlavuza ongavamile