Ngaba umntwana wakho uhlala efumana umkhuhlane, ukukhwehlela, izifo zendlebe, okanye izifo zolusu? Ngaba isifo esinye siyaphucuka ngaphambi kwesinye? Ngokwesiqhelo, sicinga ukuba amandla okuzikhusela komntwana aphantsi kancinci kwaye ufumana izifo esikolweni nakwiindawo zokudlala. Nangona kunjalo, kwiimeko ezingaqhelekanga, oku kunokubangelwa yimeko ethile yemfuza ebizwa ngokuba yi-WHIM Syndrome . Nangona oku kungathethwa kakhulu ngako, kubaluleke kakhulu ukukwazi.
Yintoni le WHIM syndrome?
Ngamafutshane, i-WHIM syndrome sisifo esingaqhelekanga kakhulu esichaphazela amajoni omzimba wethu. Igama elithi WHIM livela kwiileta zokuqala zeempawu ezine eziphambili zesifo.
- Iintsumpa (iintsumpa)
- I-Hypogammaglobulinemia (amanqanaba aphantsi ee-antibodies egazini)
- Izifo (izifo ezixhaphakileyo)
- Ukopha kwe -Myeloalveolar (ukuxinana kweeseli ezimhlophe zegazi kumongo wethambo)
Khawucinge ukuba umzimba wethu unomkhosi weeseli ezimhlophe zegazi. Umsebenzi wala majoni kukulwa neentshaba ezifana neebhaktheriya kunye neentsholongwane ezibangela izifo. Kumntu onesifo se-WHIM syndrome, amajoni abaluleke kakhulu kulo mkhosi, iiseli ezimhlophe zegazi ezibizwa ngokuba zii-neutrophils , zinamathela kwisiseko sazo, umongo wethambo. Azikwazi ukuphuma, ziye egazini, ukuze zilwe nezifo. Ngoko ke, inkqubo yokuzikhusela yomzimba ibuthathaka, kwaye baqala ukugula rhoqo.
Kunqabile kangangokuba kukholelwa ukuba kuchaphazela umntwana omnye kuphela kwizigidi ezi-5 ezizelweyo, ngoko kusenokwenzeka ukuba awukaze uve ngayo ngaphambili.
Zithini iimpawu ze-WHIM syndrome?
Ezi mpawu zinokwahluka kakhulu kumntu nomntu. Abanye abantu baneempawu ezibuthathaka kakhulu. Abanye banokufumana usulelo olunzima olunokuba yingozi ebomini. Ezi mpawu zihlala ziqala ebuntwaneni, kodwa ngamanye amaxesha isifo sinokungafunyaniswa de kube mdala.
| Uhlobo oluneempawu | Izinto ohlala uzibona |
|---|---|
| Iimpawu zokuqala ebuntwaneni |
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| Iimpawu ezinokwenzeka kamva |
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Ingakumbi malunga neentsumpa kunye nomngcipheko womhlaza
Abantu abaninzi abasingqongileyo banokosulelwa yi-human papillomavirus (HPV), ebangela iintsumpa. Nangona kunjalo, kumntu onenkqubo yomzimba yokuzikhusela esempilweni, le ntsholongwane iyanyamalala emzimbeni phantse ngokuzenzekelayo. Nangona kunjalo, ngenxa yokuba inkqubo yomzimba yomntu one-WHIM syndrome ibuthathaka, kunzima ukulwa nale ntsholongwane ye-HPV. Ke ngoko, le ntsholongwane ihlala emzimbeni ixesha elide, ibangela iintsumpa, kwaye kwezinye iimeko, inyusa umngcipheko womhlaza.
Yintoni ebangela oku? Ingaba oku kuyasulela?
Eyona nto ibangela i-WHIM syndrome kukuguquka kwejini ebizwa ngokuba yi-'CXCR4' emzimbeni wethu. Cinga ngale jini njengenkqubo efundisa iiseli zethu. Xa le jini itshintsha, iiseli zegazi ezimhlophe ezikhankanyiweyo apha ngasentla (iiNeutrophils) aziphumi kumongo wethambo.
Ngenxa yokuba esi sisifo semfuza, asinakusasazeka ukusuka komnye umntu ukuya komnye ngokuthimla okanye ngokuchukumisa . Nangona kunjalo, sinokudluliselwa ukusuka kubazali ukuya ebantwaneni. Ukuba umzali ngamnye unale nguqu yemfuza, umntwana ngamnye abanaye unethuba lama-50% lokufumana esi sifo njengelifa .
Ungayixilonga njani ngokuchanekileyo le sifo?
Ngenxa yokuba esi sisifo esingaqhelekanga, kunzima ukusifumanisa ngovavanyo olunye kuphela. Ugqirha wakho uza kukuhlola ngononophelo iimpawu zakho nezomntwana wakho, ukuba bazifumana kangaphi izifo, aze emva koko acebise ukuba kwenziwe uvavanyo oluhlukeneyo.
- Ubalo lweGazi olupheleleyo (CBC): Oku kujonga ukuba iiseli ezimhlophe zegazi ziphantsi na, ingakumbi ii-neutrophils, egazini.
- Uvavanyo lwe-antibody (uvavanyo lwegazi lwe-Immunoglobulin G - IgG):Olu vavanyo lwenziwa ukuze kubonwe ukuba amanqanaba ee-antibodies ezilwa nosulelo aphantsi na.
- I-Bone Marrow Biopsy: Kule nkqubo, kuthathwa isampuli encinci yomongo wethambo kwithambo lesinqe phantsi kwe-anesthesia ethambileyo kwaye ihlolwe phantsi kwe-microscope. Oku kunokuqinisekisa ngokuchanekileyo ukuba iiseli zegazi ezimhlophe zibambeke kwi-bone marrow (myelokathexis).
- Uvavanyo lweMfuzo: Olu vavanyo lunokuqinisekisa ukuba kukho utshintsho kwi-gene ye-`CXCR4` ebangela i-WHIM syndrome.
Okukhona isifo sifunyanwa ngokukhawuleza, kokukhona amathuba okuba sifumaneke sisifo ancipha, njengokulaliswa esibhedlele rhoqo, ukulahlekelwa kukuva, kunye nokonakala kwemiphunga.
Ziziphi iindlela zonyango lwe-WHIM syndrome?
Akukho nyango lwale meko okwangoku. Nangona kunjalo, kukho iindlela ezahlukeneyo zonyango ezinokunceda ukulawula iimpawu, ukukhusela kwiintsholongwane, kunye nokukunceda uphile ubomi obuqhelekileyo.
Iintlobo zamachiza
- I-Xolremdi: Eli liyeza elitsha elivunyiweyo ngo-2024. Linceda ukukhulula iiseli ezimhlophe zegazi ezibambeke kumongo wethambo. Oku kunokunciphisa umngcipheko wosulelo malunga ne-40%.
- Unyango lwe-G-CSF: Olu luhlaselo. Luvuselela umongo wethambo kwaye luncede ekuveliseni iiseli ezimhlophe zegazi ezingaphezulu.
- Unyango lwe-IgG: Kwabo banezinga eliphantsi le-antibody, inkqubo yomzimba yokuzikhusela iqiniswa ngokunikezelwa kwee-antibodies ezivela ngaphandle.
- Ii-antibiotics: Xa kuvela izifo ezibangelwa ziintsholongwane (ezifana ne-pneumonia, izifo zolusu), ugqirha wakho uya kukunika ezi ziyobisi ukuze azilawule.
Unyango lweentsumpa
Ukuba iintsumpa zivela rhoqo, ungabonana nogqirha wesikhumba ukuze azisuse. Kukho iindlela ezahlukeneyo zokwenza oko.
- Utyando lweCryosurgery
- Utyando lwe-Electrosurgery
- Unyango lwe-laser
- Iintlobo zeengubo ezikhethekileyo
Ungaphila njani usempilweni xa une-WHIM syndrome?
Xa uphila nesi sifo singaqhelekanga, kufuneka ucinge ngakumbi ngempilo yakho.
- Ukutya okulungileyo: Ukutya okunesondlo kubaluleke kakhulu ukugcina amasosha omzimba wakho eqinile. Faka imifuno, iziqhamo, iinkozo ezipheleleyo, intlanzi kunye nenkukhu ekutyeni kwakho.
- Ukuzilolonga: Ukuzilolonga kunceda ukugcina umzimba unamandla kwaye kwandisa amajoni omzimba. Yenza into oyithandayo, njengomdlalo, ukuhamba, okanye ukudada, yonke imihla.
- Ukhuseleko kwizifo:
- Ziphephe iindawo ezinabantu abaninzi kangangoko unako.
- Hlamba izandla zakho rhoqo ngesepha.
- Thetha nogqirha wakho uze wenze isigqibo sokuba zeziphi izitofu ozifunayo wena nosapho lwakho.Thetha ngokukodwa ngezinto ezifana nogonyo lwe-HPV, ugonyo lwe-pneumonia, kunye nogonyo lomkhuhlane lwaminyaka le.
Ukongeza kugqirha wosapho lwakho, ungadinga uncedo loogqirha abahlukeneyo, njengogqirha wezifo zomzimba, ingcali yegazi, kunye nogqirha wesikhumba, ukuze bakunyange.
Umyalezo Wokuya Ekhaya
- I-WHIM syndrome yimeko engaqhelekanga kakhulu, eyenzeka ngokwemfuza. Asikuko ukuba ityala lakho okanye lomntwana wakho libangelwa yiyo.
- Ukuba umntwana wakho ufumana usulelo olungaqhelekanga, kubaluleke kakhulu ukuthetha nogqirha ngalo mba ngaphandle kokungakhathali .
- Esi sifo asisuleli, kodwa sinokudluliselwa kubazali ukuya ebantwaneni.
- Nangona kungekho nyango lupheleleyo loku, unyango lwangoku lunokulawula iimpawu, lunciphise iingxaki, kwaye lugcine umgangatho wobomi ulungile.
- Thetha ngokukhululekileyo nogqirha wakho malunga naziphi na iingxaki okanye iingxaki zempilo onokuba nazo.
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