Ngaba wena okanye umntu omaziyo uhlala egula? Ngaba ufumana umkhuhlane, umkhuhlane, usulelo lwendlebe, usulelo lwemiphunga, njl.njl., uze emva koko kuqale olunye? Ngamanye amaxesha kunokubakho isizathu esinzulu emva kwezi zinto esingacingi ngaso. Namhlanje sithetha ngemeko yemfuza engaqhelekanga kakhulu, kodwa enokuba yingozi kakhulu. Oku kubizwa ngokuba yi-WHIM syndrome.
Ngamafutshane, yintoni le WHIM syndrome?
I-WHIM syndrome sisifo semfuza esichaphazela amajoni omzimba wethu, nto leyo ewenza buthathaka amandla omzimba okulwa nesifo. Ayiqhelekanga kakhulu. Igama elithi WHIM livela kwiileta zokuqala zeempawu ezine eziphambili zesifo. Asinguye wonke umntu onazo zonke ezi mpawu zine, kodwa ezi zezona zixhaphakileyo.
Makhe sibone ukuba zeziphi iimpawu ezichazwa zezi leta zine.
| Ileta | Intsingiselo kunye nengcaciso elula |
|---|---|
| W - Iintsumpa | Iintsumpa: Ngenxa yokuba aba baguli banenkqubo yomzimba yokuzikhusela ephantsi, intsholongwane ebizwa ngokuba yiHuman Papillomavirus (HPV) inokungena ngokulula emzimbeni ize ibangele iintsumpa eluswini nakwizitho zangasese. Le ntsholongwane yeHPV ikwanyusa umngcipheko wokufumana iintlobo ezithile zomhlaza. |
| H - I-Hypogammaglobulinemia | Amanqanaba aphantsi e-Antibody (Hypogammaglobulinemia): Ngamafutshane, ii-antibodies zifana namajoni alwa neentsholongwane ezingena emizimbeni yethu. Aba baguli banezinga eliphantsi lale antibodies emizimbeni yabo. Oku kungenxa yokuba baneeseli ezimhlophe ezimbalwa zegazi ezibizwa ngokuba zii-B cells ezivelisa ii-antibodies. Ngoko ke, banamathuba amaninzi okufumana usulelo. |
| I - Iintsholongwane | Usulelo: Ngenxa yobuthathaka bomzimba, aba bantu badla ngokufumana usulelo . Ingakumbi usulelo lwemiphunga, lwendlebe nolusu luxhaphakile. |
| M - Myelokathexis | I-Myelokathexis: Le yeyona ngxaki iphambili neyona ikhethekileyo yesi sifo. Iiseli ezimhlophe zegazi ezilwa neentsholongwane emizimbeni yethu ziveliswa kumongo wethambo. Kufana namajoni aqeqeshwa kwinkampu yomkhosi. Kodwa kubantu abanesi sifo, ezo seli ezimhlophe zegazi azikwazi ukuphuma kumongo wethambo ziye kwindawo yosulelo. Zibanjwa ngaphakathi kumongo wethambo. Ngoko ke, xa kuvela usulelo, iiseli azizi ukulwa nalo. |
Okubalulekileyo kukuba, ayizizo zonke izigulana ezine-WHIM syndrome eziba nazo zonke ezi mpawu zine. Ezinye zinokuba neempawu ezincinci kakhulu, ngelixa ezinye zinokuba nezifo ezixhaphakileyo nezinzima ezinokuba yingozi ebomini.
Isebenza njani iXolremdi (mavorixafor)?
Njengoko besitshilo ngaphambili, iiseli zegazi ezimhlophe zezi zigulana zinamathela kumongo wethambo, akunjalo? Isizathu soku kukuguquka kwejini ebizwa ngokuba yi-'CXCR4' emzimbeni wethu. Le jini ye-'CXCR4' yenza iproteni ekhethekileyo ebizwa ngokuba yi-'CXCR4' receptor. Cinga ngale receptor njengomlindi wesango apho iiseli zegazi ezimhlophe zishiya umongo wethambo.
Kubantu abane-WHIM syndrome, ngenxa yolu tshintsho kwi-gene ye-'CXCR4', loo mgcini-sango akasebenzi kakuhle. Ugcina isango livaliwe. Ngoko ke iiseli ezimhlophe zegazi azikwazi ukuphuma.
Iyeza elitsha elibizwa ngokuba yiXolremdi (mavorixafor) lisebenza ngokuvala i-receptor ye-CXCR4 engasebenzi kakuhle. Kufana nokunika umgcini-sango ithuba lokuvula ucango. Emva koko, iiseli zegazi ezimhlophe ezazivaleleke kumongo wethambo zikhululekile ukungena egazini kwaye ziye apho zifuna ukulwa nosulelo khona. Eli liyeza lokuqala elavunywa ngo-2024 ukunyanga unobangela oyintloko we-WHIM syndrome.
Ndingayisebenzisa njani le yeza?
I-Xolremdi yikhaphu oyisela ngomlomo kanye ngemini. Ifanele ithathwe ungenanto esiswini . Eyona ndlela ilungileyo yokuyisela kukuzila ukutya ubusuku bonke emva kwesidlo sangokuhlwa uze uyisele kusasa. Akufanele utye okanye usele nantoni na enye ubuncinane imizuzu engama-30 emva kokusela iyeza. Umthamo owufunayo uya kuxhomekeka kubunzima bakho, ngoko ke ugqirha wakho uya kugqiba.
Impumelelo yale mayeza yavavanywa njani?
Kuye kwenziwa uphando lwezonyango ukuvavanya ukhuseleko kunye nokusebenza kakuhle kwale chiza. Lubandakanye izigulane ezingama-31 ezine-WHIM syndrome ezazinotshintsho lwe-CXCR4 gene oluqinisekisiweyo.
Malunga nesiqingatha sabathathi-nxaxheba kolu phononongo yayingabantu abaselula abaphakathi kweminyaka eli-12 neli-17. Bahlulwe baba ngamaqela amabini, elinye iqela lanikwa iyeza iXolremdi, kwaye elinye iqela lanikwa i-placebo yonke imihla.
Abaphandi bajonge kakhulu inani lohlobo lweseli emhlophe yegazi ebizwa ngokuba yi-"neutrophils" egazini. Balinganise ukuba eli nani lihlale ixesha elingakanani kwinqanaba elithile elikhuselekileyo (iiseli ezingama-500/microliter).
Iziphumo ziyakhuthaza kakhulu:
- Abo bathathe iXolremdi babenamanqanaba e-neutrophil awayekwinqanaba elikhuselekileyo malunga neeyure ezili-12 ngosuku kunabo bangazange bathathe.
- Kwakhona, izinga losulelo lonyaka kwabo bafumana unyango lehle laya ngaphantsi kwesibini ngonyaka , ngelixa kwabo bangafumananga unyango lenyuka laya kutsho ngaphezulu kwesine ngonyaka.
Ezi ziphumo zibonise ukuba i-Xolremdi inciphise kakhulu umngcipheko wokosuleleka ngokukhupha iiseli ezimhlophe zegazi. Nangona kunjalo, khumbula ukuba nangona kukho ezi ziphumo zovavanyo, impendulo yomzimba wakho inokwahluka.
Ngubani ongafanele asebenzise iXolremdi?
Eli yeza alifanelekanga ukusetyenziswa ukuba unesifo esibi sezintso okanye sesibindi, ukhulelwe okanye uncancisa. Kwakhona, eli yeza linokusebenzisana namanye amayeza. Kungoko ke, kubalulekile ukuthetha nogqirha wakho ukuze ugqibe ukuba iXolremdi ikulungele na.
Zithini iindlela ezisebenzisana ngayo iziyobisi nezinye iziyobisi?
Le yinxalenye ebaluleke kakhulu. I-Xolremdi inokubangela iingxaki xa idityaniswe namanye amayeza owasebenzisayo.
- Ukusebenzisa i-Xolremdi kunye namayeza athile (umz., i-`P-gp` okanye i-`CYP3A4` inhibitors) kunokunyusa imiphumo ye-Xolremdi kwaye kwandise umngcipheko wemiphumo emibi.
- Amanye amayeza (umz., ii-inducers ezinamandla ze-`CYP3A4`) anokunciphisa ukusebenza kwe-Xolremdi.
- Kwakhona, i-Xolremdi inokunyusa imiphumo yezinye iziyobisi ozisebenzisayo (`CYP2D6`, `CYP3A4` okanye `P-gp` substrates).
- Ngokukodwa, kufuneka uphephe ukusebenzisa iXolremdi kunye namanye amayeza anokubangela utshintsho oluyingozi kwisantya sentliziyo (`i-QT prolongation`).
Olu luhlu aluphelelanga. Ngoko ke, ngaphambi kokuba uqale iXolremdi, qiniseka ukuba uxelela ugqirha wakho ngayo yonke imithi oyithathayo - kuquka amayeza kagqirha namayeza athengiswa ngaphandle kwemvume kagqirha, iivithamini, amayeza esintu, kunye namayeza e-Ayurvedic.
Ungazilawula njani iziphumo ebezingalindelekanga xa zisenzeka?
Njengayo nayiphi na imithi, iXolremdi inokuba nemiphumo emibi. Le yeyona ixhaphakileyo.
| Imiphumela | Oko unokukwenza |
|---|---|
| Inani eliphantsi leeplatelet egazini (`Thrombocytopenia`) | Oku kunokubangela ukudumba okanye ukopha okulula. Ukuba oku kuyenzeka, yazisa ugqirha wakho ngokukhawuleza. |
| Ukuqhambuka kwesikhumba | Ungabona iimpawu ze-'pityriasis', ezinokwenza ulusu lwakho lome kwaye lube namaxolo. Xelela ugqirha wakho ngale nto. |
| Ukopha empumlweni | Ukuba unesifo sokopha impumlo, hlala uthe nkqo uze usebenzise ubhontsi wakho nomnwe wakho wokukhomba ukuze udibanise indawo ethambileyo yempumlo yakho. Ukuba ukopha akuyeki emva kwemizuzu engama-20 okanye ukuba kukhulu, funa ingcebiso kagqirha ngoko nangoko okanye uye kwiSebe leeNgxamiseko elikufutshane (ETU). |
| Ukuhlanza | Ukuba uyahlanza, sela ulwelo oluninzi. Musa ukutya okuninzi ngaxeshanye, yitya ukutya okuncinci nokulula. |
| Isiyezi | Ukuba nesiyezi kunokubangela ukuwa. Ngoko ke, kuphephe izinto ezifana nokujika intloko yakho ngequbuliso okanye ukuvuka ngequbuliso uhleli okanye ulele. |
Ezi azizo zonke iziphumo ebezingalindelekanga ezinokubakho. Ukuba uneempawu ezikukhathazayo okanye ezikwenza ungakhululeki, qiniseka ukuba uthetha nogqirha wakho ukuze akunike iingcebiso zonyango.
Oko kufuneka ukwazi xa usebenzisa eli yeza
I-Xolremdi ithathwa njenge "yeza elikhethekileyo." Oku kuthetha ukuba ayisiyonto unokuyithenga kwikhemesti eqhelekileyo. La ngamayeza abiza kakhulu asetyenziselwa ukunyanga izifo ezingaqhelekanga nezintsonkothileyo.
Ukuba unikwe eli yeza, ingcali yakho yokunyanga iya kukuchazela indlela yokulifumana kunye nekhemesti yesibhedlele oza kulifumana kuyo. Le nkqubo inokuba nzima kancinci, kodwa ugqirha wakho kunye nabasebenzi besibhedlele baya kukunika ulwalathiso olufunekayo.
Umyalezo Wokuya Ekhaya
- I-WHIM syndrome sisifo esingaqhelekanga esichaphazela inkqubo yomzimba yokuzikhusela, esibangela usulelo oluqhelekileyo.
- I-Xolremdi (mavorixafor) liyeza elitsha elinyanga unobangela wesi sifo, linceda ukukhulula iiseli ezimhlophe zegazi ezivaleleke kumongo wethambo.
- Le yikhaphu ekufuneka ithathwe yonke imihla esiswini esingenanto. Kuphephe ukutya imizuzu engama-30 emva kokusela ipilisi.
- Ukuze uphephe iziphumo ebezingalindelekanga eziyingozi, kubalulekile ukwazisa ugqirha wakho ngazo zonke ezinye iziyobisi (kuquka iivithamini kunye namayeza esintu) ozisebenzisayo.
- Ukuba ufumana isiphumo esibi kakhulu, njengokuphuma kwegazi elininzi, fowunela ugqirha wakho ngoko nangoko okanye uye kwiSebe leNgxamiseko (ETU) elikufutshane.
- Ekubeni eli liyeza elikhethekileyo, ugqirha wakho uza kukhokela kwinkqubo yokulifumana.
💬 Comments (0)
Akukho zimvo zithunyelweyo okwangoku. Faka uluvo lwakho apha okokuqala.
Faka uluvo lwakho