Isifo sikashukela sohlobo 1 siseyinye yezifo ezivame kakhulu izifo ezingamahlalakhona eziyinkimbinkimbi kakhulu Kunzima ukukuphatha ebuntwaneni nasebusheni, kodwa futhi nasebudaleni. Nakuba kuvame ukuhlotshaniswa kuphela "noshukela ophezulu egazini," empeleni kuyinqubo yokuzivikela komzimba eqala isikhathi eside ngaphambi kokuba kuvele izimpawu zokuqala futhi ihilela isimiso somzimba sokuzivikela, izakhi zofuzo kanye nendawo ezungezile.
Eminyakeni yamuva nje, ucwaningo eSpain nakwamanye amazwe aseYurophu luveza imiphumela emihle kakhulu. ushintsho olukhulu endleleni yendabukoUkugxila kushintshela ekuxilongeni lesi sifo kuphela lapho i-pancreas isivele yonakele kakhulu kuya ekuzameni ukuyikhomba ezigabeni zayo ezithule, ukuhlola izimpawu zokukhulelwa, kanye nokuhlola imithi ekwazi ukulibazisa ukuqala kwezokwelapha. Konke lokhu kwenzeka ngaphandle kokukhohlwa umthelela wansuku zonke wokuphila nesimo esidinga izinqumo eziqhubekayo, imini nobusuku.
Kuyini isifo sikashukela sohlobo 1 futhi kungani sivela?
Kusifo sikashukela sohlobo 1, amasosha omzimba ihlasela futhi ibhubhise amaseli e-beta e-pancreasokuyimbangela yokukhiqiza i-insulin. Ngaphandle kwe-insulin eyanele, i-glucose ayikwazi ukungena kahle emaseli futhi iqongeleleke egazini, okubangela i-hyperglycemia futhi, uma ingelashwa, izinkinga ezinkulu njenge-ketoacidosis yesifo sikashukela.
Ngokungafani nesifo sikashukela sohlobo 2, lapho indlela yokuphila idlala indima enkulu, isifo sikashukela sohlobo 1 sibhekwa njengesifo sikashukela. isifo sokuzivikela komzimba esisekelwe ku-genetic okuthonywa yizici zemvelo. Kulinganiselwa ukuthi izakhi zofuzo ze-HLA zesigaba II zinegalelo cishe ku-40-50% wengozi yezakhi zofuzo, kodwa azichazi zonke izimo: abanye abantu bahlakulela isifo sikashukela sohlobo 1 ngaphandle kokuba nephrofayili yezakhi zofuzo eyingozi kakhulu, okubonisa ukuthi kusekhona izingcezu ezingekho kule nkinga.
Izifundo zisikisela ukuthi ziyabandakanyeka izici ezahlukahlukene zemveloLezi zinto zifaka phakathi ukutheleleka ngamagciwane ebuntwaneni, ukutheleleka kokuphefumula noma kokugaya ukudla ngesikhathi sokukhulelwa, izehlakalo zokuphila ezicindezelayo kakhulu, noma ukuchayeka kumakhemikhali athile aqhubekayo. Akukho neyodwa yalezi zinto "edala" lesi sifo, kodwa zingalawula ubuthakathaka bamaseli e-beta ekuhlaselweni amasosha omzimba.
ESpain, imiphakathi yesayensi isikhumbuza ukuthi isifo sikashukela sohlobo 1 singavela kunoma yimuphi ubudala, yize sibonisa amazinga amabili acacile okutheleleka ezinganeni nasebancanePhakathi kweminyaka engu-4 nengu-7, kanye naphakathi kweminyaka eyi-10 nengu-14. Noma kunjalo, kulinganiselwa ukuthi ngaphezu kwengxenye yokuxilongwa kwenziwa lapho umuntu esemdala, okudinga ukuba kugcinwe izinga eliphezulu lokusola ngisho nangaphandle kwezingane.
Izigaba zesifo: isigaba sokuthula ngaphambi kokuqala
Eminyakeni eyishumi edlule, kuye kwahlanganiswa imodeli esekelwe esiteji esiza ukuqonda kangcono ukuvela kwesifo sikashukela sohlobo 1. Lolu hlelo lusenza sikwazi ukukhuluma ngalesi sifo isikhathi eside ngaphambi kokuba izimpawu zivele, into ebalulekile ekuqondiseni ukuhlolwa kanye nokwelashwa okusha.
Esigabeni soku-1, amasosha omzimba aseqalile ukubona nokubhubhisa amaseli e-beta, kodwa ama-pancreas asakhiqiza i-insulin eyanele ukugcina amazinga kashukela egazini ajwayelekile. Kulesi sigaba, ukuhlolwa kwegazi kuyathola ama-autoantibodies amabili noma ngaphezulu aqondile ngokumelene nezakhiwo zamaseli e-beta, kodwa umuntu uzizwa ephilile futhi akaqapheli lutho olungavamile.
Isigaba sesi-2 sibonakala ngokuba khona kwala ma-autoantibodies afanayo, kodwa sihambisana izinguquko emazingeni kashukela ezingasafaneleki ngaphakathi kwebanga elijwayelekile, yize kungekho zimpawu ezicacile. Lokhu kwaziwa ngokuthi i-dysglycemia: i-pancreas iqala ukuba nobunzima bokuhlangabezana nezidingo ze-insulin.
Isigaba sesi-3 siphawula ukuqala kwesifo sikashukela sohlobo 1. Kulesi sigaba, sekuvele kukhona ukulahlekelwa okukhulu kakhulu kwamaseli e-beta Bese kuvela izimpawu ezivamile: ukoma ngokweqile, ukwanda kokuchama, ukukhathala okukhulu, ukwehla kwesisindo okukhulu, ngisho ne-ketoacidosis yesifo sikashukela, okungadinga ukungeniswa e-ICU. Kuleli qophelo, okuwukuphela kwendlela yokwelapha ukuqala i-insulin, kungaba ngokujova kaningi nsuku zonke noma ngamaphampu e-insulin.
Kusukela ngaleso sikhathi kuqhubeke, lesi sifo singena esigabeni esimisiwe, lapho umgomo uwukuthi ukufeza ukulawulwa okuqinile kwe-glycemic ukunciphisa ingozi yezinkinga zesikhathi eside. Noma kunjalo, isizathu esiyinhloko salo lonke inqubo yokuzivikela komzimba asikacaci, futhi ucwaningo luyaqhubeka nokufuna ukuthi yini ebangela ukuhlaselwa kwamasosha omzimba nokuthi kungamiswa kanjani ngesikhathi.
Ukuhlolwa komndeni kanye nokutholakala kusenesikhathi eSpain
Ulwazi ngalezi zigaba luholele ekuthuthukisweni izinhlelo zokuhlola kubantu abasengozini enkuluikakhulukazi izihlobo zezinga lokuqala zeziguli ezinesifo sikashukela sohlobo 1, ezingozi yazo iphakathi kwezikhathi eziyi-15 nezingama-20 eziphakeme kuneyabantu abavamile.
Ngokwesibonelo, eGalicia, izibhedlela ezifana ne-University Clinical Hospital of Santiago (CHUS) kanye ne-Vigo Hospital (Chuvi) zisebenzise amasu okuthola phakathi kwamalungu omndeni, isinyathelo esibonisa indlela I-Galicia ikhuthaza ukulawula isifo sikashukela ngobuchwepheshe nokuqeqeshwa.
Odokotela be-endocrinologists bezingane babonisa ukuthi izinhlobo ezine eziyinhloko zama-autoantibodies ziyahlolwa njengezihlobene nengozi eyengeziwe yokuthola lesi sifo. Ukuba khona kwe-antibody eyodwa kungase kuhlale kungashintshi iminyaka noma, kwezinye izimo, ama-antibodies amasha anganqwabelana kuze kube yilapho kuhlangatshezwana nezindinganiso zesifo sikashukela sohlobo 1 sangaphambi kokulashwa.
Lolu hlobo lokuhlolwa, ngaphezu kokunciphisa inani lokuqala okubi kakhulu—njenge-ketoacidosis egcina i-ICU—luvula ithuba ifasitela lemfundo nokulungiselela Emindenini: kubavumela ukuthi bafunde ngokuthula ukuthi bangaphatha kanjani i-insulin, baqonde indima yokudla, ukuzivocavoca kanye nokwehla kwe-hypoglycemia, futhi bajwayelane nobuchwepheshe obufana nezinzwa zokuqapha ushukela njalo.
Ngesikhathi esifanayo, kuthathwa izinyathelo zokwandisa ukuhlolwa ngale kwamalungu omndeni. E-Basque Country, ucwaningo lokuhlola lwe-SCREEND1A, olukhuthazwe yi-Osakidetza kanye ne-Biobizkaia Health Research Institute, luyaqhubeka ukuhlola ukukwazi ukuhlola izingane ezijwayelekile phakathi kweminyaka emi-3 neyi-13 ubudala ezikhungweni zezempilo. Inhloso: ukuqinisekisa ukuthi uhlelo lwabantu luzovumela yini ukutholakala kwamacala amaningi ezigabeni 1 nesesi-2 kanye nokuhlola izindleko zalo kanye nomthelela wangempela.
Izimpawu egazini le-umbilical cord: inqubo ingaqala ngaphambi kokuzalwa
Ngaphandle kokuhlolwa kwezingane, amanye amaqembu ocwaningo aseYurophu naseMelika ahlaziya ukuthi kungenzeka yini ukuthola izimpawu zengozi yesifo sikashukela sohlobo 1. kusukela ekuzalweniUcwaningo oluhlanganyelwe olwenziwe ngamayunivesithi aseLinköping (eSweden) naseFlorida (e-United States), olushicilelwe ephephabhukwini i-Nature Communications, lugxile ekutadisheni igazi le-umbilical cord.
Kulo msebenzi, oyingxenye yocwaningo lweqembu elithi "All Babies in Southeast Sweden" (ABIS), kuhlolwe amasampula egazi kusukela izingane ezisanda kuzalwa ezingaphezu kuka-16.000 eqoqwe phakathi kweminyaka yama-1990 nasekuqaleni kwawo-2000. Ngokuhamba kwesikhathi, izingane ziye zalandelwa ngemininingwane yezokwelapha neyebhayoloji ukuze kutholakale ukuthi ubani othole isifo sikashukela sohlobo 1 noma cha.
Besebenzisa amasu okufunda komshini, abacwaningi bathole i- iphethini yamaprotheni okuvuvukala kanye nokuzivikela komzimba egazini lentambo, okwavela kaningi kulabo abathola lesi sifo kamuva. Ngokusho kwababhali, le "phrofayili yamaprotheni" ingabikezela ingxenye enkulu yamacala esikhathi esizayo, ngaphandle kwengozi yezakhi zofuzo yakudala.
Okutholakele kusikisela ukuthi izinqubo zokuvuvukala kanye nokucindezeleka kwamaselula Izici ezenza abantu bangakwazi ukuzivikela ngokuzimela zingase ziqale kusukela ekuqaleni kokukhulelwa. Ngaphezu kwalokho, kuye kwaphawulwa ukuthi amanye amaprotheni ahilelekile angathonywa ukuchayeka kukamama kumakhemikhali aqhubekayo, njengezakhi ezithile ze-perfluoroalkyl (PFAS).
Abacwaningi bagcizelela ukuthi inhloso yabo akuyona ukwethula uhlelo lokubikezela lomuntu ngamunye ngokushesha, kodwa ukusisiza siqonde kangcono indlela i-biology yengozi ehlelwe ngayo ezigabeni zokuqala kakhulu nokuthi yiziphi izici zemvelo ezingashintshwa ukuze kuncishiswe ukwanda kwesikhathi esizayo. Enye yezinzuzo zale ndlela ukuthi igazi le-umbilical cord liyizicubu ezilahlwayo ezimweni eziningi futhi ezingasetshenziswa ngaphandle kwezinqubo zokuhlasela zosana olusanda kuzalwa.
Imithi emisha enciphisa inqubekela phambili: indima ye-teplizumab
Kuze kube muva nje, ukwelashwa kwesifo sikashukela sohlobo 1 kwakunqunyelwe kuphela ukuphathwa kwe-insulinngezinhlobo ezahlukene zemithi namadivayisi, kodwa ngaphandle kwemithi ekwazi ukuguqula ngqo inkambo yokuzivikela komzimba. Lokhu kwaqala ukushintsha ngokuvunyelwa yi-European Medicines Agency (EMA) kwe-teplizumab, ethengiswa njenge-Teizeild, okuyindlela ehambisana nezinqumo ezinjengalezo. I-WHO yandisa uhlu lwayo lwemithi ebalulekile ngomdlavuza nesifo sikashukela.
I-Teplizumab iyi-antibody ye-monoclonal eqondiswe kuma-T lymphocytes, lapho inikezwa abantu abasesigabeni sesi-2—abanama-autoantibodies kanye ne-dysglycemia, kodwa azikho izimpawu zomtholampilo okwamanje—, kuboniswe ukuthi kubambezela ukuqala kwesigaba sesi-3 ngesilinganiso seminyaka emibili. Isibonakaliso esivunyelwe eYurophu sigxile ku izingane ezineminyaka engu-8 ubudala kanye nentsha ngengozi ephezulu ebhaliwe.
Lo muthi usebenza ngokunciphisa ulaka lwama-T lymphocyte ahlasela amaseli e-beta, okuvumela ukukhiqizwa kwe-insulin yangaphakathi ukuthi kugcinwe isikhathi eside. Awuvimbeli lesi sifo impilo yonke, kodwa uyafeza londoloza isikhathi esibalulekileLokhu akuyona into encane uma ucabanga ngeminyaka yeziguli eziningi ezizongenela ukhetho.
ESpain, izinyathelo zokuphatha nezokuhlela zisadingeka ukuze isetshenziswe kabanzi: kusukela ezingxoxweni zamanani kuya ekuchazeni izinqubo zokuhlonza abantu abasesigabeni sesi-2 nokuqeqesha amaqembu azoyiphatha. Noma kunjalo, amacala okusetshenziswa ngozwela asevele egunyaziwe, njengaleyo yentombazane yaseGalicia lapho kwasetshenziswa khona i-teplizumab ngokunembile ukuzama ukubambezela ukuqala komtholampilo okutholiwe ngenxa yokuhlolwa komndeni.
Okwamanje, izibhedlela ezifana ne-CHUS eSantiago kanye ne-Chuvi eVigo zihlanganyela ezivivinyweni ezihlola ukuthi ukusetshenziswa kwe-teplizumab kuyini. Isigaba sesi-3, ngemva nje kokuxilongwaKuyasiza ukwandisa isikhathi esibizwa ngokuthi "i-honeymoon"—leso sikhathi ngemva kokuqala kwe-insulin lapho i-pancreas isenayo imali egciniwe futhi kudingeka imithamo ephansi—futhi kuthuthukisa ukulawulwa kwe-glycemic uma kuqhathaniswa neqembu elithola i-placebo.
Ukuphila ngesifo sikashukela sohlobo 1: isifo esinzima kakhulu
Ngaphandle kwedatha kanye nokwelashwa okusha, iqiniso lansuku zonke lesifo sikashukela sohlobo 1 lisalokhu likhona imindeni ifuna kakhuluAbanakekeli bachaza umuzwa wokuba "usesimweni" amahora angama-24 ngosuku: ukubala ama-carbohydrate, ukulungisa i-insulin, ukuqapha ukuzivocavoca, ukuphendula kuma-alamu ezinzwa, kanye nokwenza izinqumo njalo ngaphandle kwesikhala sephutha.
Odokotela be-endocrinologists bezingane bachaza ukuthi ukwelashwa nge-insulin kuhilela ukufaka esikhundleni somsebenzi we-pancreas Lokhu kufezwa ngokujova okuningana ngaphansi kwesikhumba ngosuku noma amaphampu okufaka axhunywe ezinhlelweni zokuqapha ushukela njalo. Lobu buchwepheshe buthuthukise ikhwalithi yokuphila nokuphepha, kodwa abuqedi isidingo sokufunda nokunakekela okuqhubekayo.
Imindeni eye yabhekana nokuqala okubi kakhulu iqokomisa ubuhlungu obungokomzwelo balesi simo isifo sikashukela i-cetoacidosis kanye nokwamukelwa esibhedlela esibucayi. Yingakho labo abaye badlula kulolo lwazi bevame ukusekela kakhulu izinhlelo zokuhlola kanye nokuthola kusenesikhathi: bakhetha ukwazi kusenesikhathi ukuthi kukhona ingozi futhi babe nesikhathi sokuzifundisa nokuhlela, noma ngabe ukuxilongwa okusemthethweni kuza eminyakeni eminingi kamuva.
ESpain, izinhlangano zabantu abanesifo sikashukela kanye nabazali bezingane ezinesifo sikashukela sohlobo 1 seziye zaba abalingani abakhulu ukwabelana ngolwazi olusebenzayo, ukunikeza ukwesekwa okuhlangene, ukusiza ngezinqubo zesikole nezempilo, kanye nokunikeza izwi ezidingweni zaleli qembu phambi kohulumeni.
Ngokwezibalo, idatha evela ku-World Diabetes Atlas ilinganisela ukuthi kungaba nabantu abangaba ngu-118.000 abanesifo sikashukela sohlobo 1 eSpain, yize inani eliqondile kunzima ukulithola ngoba izindawo zokubhalisa zikazwelonke azihlukanisi kahle njalo izinhlobo ezahlukene zesifo sikashukela. EGalicia, isibonelo, kulinganiselwa ukuthi izingane eziphakathi kuka-60 no-70 ezingaphansi kweminyaka engu-15 zitholakala minyaka yonke, nokuthi Izingane eziphakathi kuka-1.500 no-2.000 zingaphila nalesi sifo., umthwalo omkhulu ohlelweni lwezempilo kanye nasemindenini.
Izinkinga ezingaziwa kakhulu: i-diabetesic cheiroarthropathy
Uma ukulawulwa kwe-glycemic kuphansi iminyaka eminingi, amathuba okuba nesifo sikashukela ayanda. izinkinga ze-microvascular njenge-retinopathy, i-nephropathy, noma i-peripheral neuropathy. Okungaziwa kakhulu, kodwa okubalulekile, ukuhamba okulinganiselwe kwamalunga ezandla, okubizwa nangokuthi i-diabetesic cheiroarthropathy.
Kuye kwachazwa amacala entsha enesifo sikashukela sohlobo 1 esihlala isikhathi eside kanye amazinga aphezulu kakhulu e-glycosylated hemoglobin Baqala ukuqaphela, kancane kancane, ukuqina okungenabuhlungu ezandleni zombili, ubunzima bokugoba nokwelula iminwe yabo, kanye nesikhumba esiqinile nesiqinile ngemuva kwamalunga abo.
Ekuhlolweni ngokomzimba, okutholakele okuphawulekayo kufaka phakathi "uphawu lomthandazo" - ukungakwazi ukuhlanganisa izintende ngokugcwele - kanye "nophawu lwebhodi," oluqukethe ukungakwazi ukubeka izandla phansi endaweni ethile. Lezi zibonakaliso, ngaphezu kokusebenza okulinganiselwe, zingaba yi- uphawu lokuqala lwezinye izinkinga ze-microvascular, ngokusho kwezifundo ezahlukahlukene.
I-pathophysiology ihlobene nokuqongelela kwemikhiqizo yokugcina ye-glycation ethuthukisiwe, eshintsha i-collagen yesikhumba kanye nezicubu ezixhumeneyo. Ukubonakala kwayo kuvame ukuhlotshaniswa nokulawulwa okungekuhle isikhathi eside, okuqinisa umqondo wokuthi ukugcina i-HbA1c iseduze kakhulu nomgomo ngangokunokwenzeka Akuyona nje inombolo, kodwa ukutshalwa kwezimali okuqondile ekuvimbeleni izinkinga esikhathini esiphakathi neside.
Le ndlela ihlanganisa ukuthuthukisa ukwelashwa kwe-insulin, ukwethula—lapho kungenzeka khona—ukuqapha ushukela njalo kanye nobuchwepheshe bokupompa, kanye nokwengeza ukwelashwa ngokomzimba nangokomsebenzi ukuze kuthuthukiswe ububanzi bokunyakaza nokusebenza kwezandla. Kunconywa futhi ukuthi ochwepheshe bezempilo bahlanganise ngokuhlelekile ukuhlolwa kokuhamba kwamalunga ekuhlolweni kwabantu abanesifo sikashukela sohlobo 1.
Uma kuhlanganiswa, ucwaningo lwesigaba sokuqala, izinhlelo zokuhlola eSpain naseYurophu, ukuhlolwa kwezimpawu egazini le-umbilical cord, kanye nokufika kwemithi efana ne-teplizumab kuveza isithombe lapho isifo sikashukela sohlobo 1 sitholakala khona. Akusagcini nje ngokuxilongwa uma "iveza ubuso bayo"Kodwa-ke, impilo yansuku zonke isalokhu inzima, futhi ukulawulwa okuhle kwe-glycemic kuyaqhubeka nokuba yitshe lesisekelo lokunciphisa izinkinga. Ngakho-ke, ukuhlanganisa intuthuko yesayensi, inhlangano yezempilo, kanye nokusekelwa komphakathi kuzobaluleka kakhulu ukuze lezi ntuthuko ziguqulele empilweni ephephile nelawuleka kalula kulabo abaphila nalesi sifo.
